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Paroxysmal cold haemoglobinuria (PCH) is a rare acquired cause of intravascular haemolysis in children. We are reporting an unusual case of PCH, a 7-year-old boy who presented with acute febrile illness, severe anaemia and passage of red colored urine. His initial haemolytic workup had shown positive direct agglutination test along with evidence of ongoing haemolysis with severe leukocytosis. Further evaluation for autoimmune haemolytic anaemia revealed positivity for Donath Landsteiner Antibody, hence establishing a diagnosis of PCH. During the hospital stay, the child required multiple red blood cells, crossmatched although “P” antigen untyped transfusions. Child gradually improved and became transfusion independent within one week. The child has been under regular follow-up since last six months and all his laboratory parameters are within normal limits.

The 2019 novel coronavirus (2019-nCoV) originated in Wuhan City of China. In India, first confirmed case of coronavirus disease (COVID-19) was reported on January 30, 2020 and India is presently hit by second wave of COVID-19. The aim of the present study was to evaluate bone marrow findings of COVID-19 by minimally invasive autopsies to aid in understanding pathophysiology of the disease. This prospective study was conducted at tertiary care centre of Western Rajasthan. After obtaining approval from Institute's ethics committee and consent from next of kins, minimally invasive autopsies were conducted in 37 COVID-19 deceased patients within an hour after the death. The tissue specimens were processed with standard biosafety measures. Electronic medical records were reviewed retrospectively and patients' clinical details and results of laboratory investigations were noted. In this prospective study, bone marrow biopsies were collected from 37 COVID-19 minimally invasive autopsies. Mean age of these cases was 61.8 years and male: female ratio was 2.36. Comorbidities were observed in 25 (67.5%) of all cases. Histopathological analysis revealed hypercellular, normocellular and hypocellular marrow in 5, 25 and 5 cases respectively (two biopsies were inadequate). There was marked interstitial prominence of histiocytes in 24 (68.5%) cases. Out of these, evidence of haemophagocytosis was observed in 14 (40%) cases, marked increase of haemosiderin laden macrophages in 20 (57.1%) cases. There was prominence of plasma cells in 28 (80%) cases. The present study attempted to fill the gap of dearth of literature from our country in COVID-19 autopsy studies by highlighting bone marrow findings. The data support the evidence of development of secondary haemophagocytic lymphocytosis in COVID-19 cases.

Background: COVID-19 pandemic has taken a great toll on the health care system worldwide. Along with the pandemic, there is also a concern regarding post COVID-19 complications in recovered patients. Thromboembolism (TE) has been reported as a fatal complication in recovered patients with COVID-19. There is still a great dilemma in post-discharge TE prophylaxis and its long-term benefits. Case Description: We reported three cases of post COVID-19 with complications related to both diseases as well as post discharge anticoagulant therapy. The first case is about a 60-yr-old male who developed Covid-19 pneumonia (moderate disease) and was discharged on rivaroxaban after initial improvement. 3 weeks later, the patient was readmitted with lower gastro-intestinal bleeding. The other two cases developed pulmonary thromboembolism within a span of 2-3 months (after recovered from COVID-19 pneumonia). Both these patients were not prescribed anticoagulants for TE prophylaxis. Conclusion: There is an imperative need for effective guidelines for post discharge TE prophylaxis in COVID-19.

Objectives The 2019 novel coronavirus (2019-nCoV) has spread across the globe with more than 6 lakh deaths. Clinical autopsies are important to understand the pathobiology of the disease. Materials and Methods Autopsy techniques have been modified to be minimally invasive autopsies in all COVID-19 positive cases, and tissue biopsies were sampled from lungs, liver, and bone marrow within an hour after death. Detailed histological analysis was performed in the sampled tissues, along with immunohistochemistry. Patients’ clinical records were collected. Statistical Analysis Descriptive statistics were used to summarize data. Results Of the 21 cases studied, 76.2% patients were ≥ 60 years of age, 80.9% were males, and 85.7% had co-morbidities. Histopathological analysis revealed diffuse alveolar damage (including exudative and organizing phase) in 88.9% cases. Microthrombi were seen in 44.4% cases. Additional findings include viral cytopathic changes, metaplastic change in the epithelium, intra-alveolar hemorrhage, and pulmonary edema. Liver showed centrizonal congestion with hepatocytic loss, lobular inflammation, steatosis, Kupffer cell hypertrophy, and sinusoidal neutrophilic infiltration, while significant portal infiltrate and cholestasis were absent to minimal. Bone marrow revealed hemophagocytosis in 60% cases. Conclusion Incorporation of minimally invasive autopsies provides an effective method to study the pathological findings in COVID-19 deaths in resource-constrained settings. Presence of pulmonary microthrombi in a significant number of cases supports the vascular events, apart from the characteristic diffuse alveolar damage, as an important pathogenic mechanism for lung injury in COVID-19 infections. Histopathological findings in the liver and bone marrow suggest indirect insult to these organs, related to circulatory and/or hyperinflammatory response to viral infections.

OBJECTIVE: This study aimed to use chest-X-ray (CXR)-based scores along with total leukocyte count (TLC) and neutrophil-to-lymphocyte ratio (NLR) in the prediction of coronavirus disease 2019 (COVID-19) in patients presenting with clinical features of severe acute respiratory illness (SARI). MATERIAL AND METHODS: This is a retrospective study involving all patients who presented with clinical features of SARI and who had undergone bedside chest X-ray (CXR), hemograms with TLC, NLR, and reverse transcriptase-polymerase chain reaction (RT-PCR) at our institute from May 1 to June 30, 2020. RESULTS: Of 204 patients, 115 tested RT-PCR-positive and 89 tested negative. The patients who presented with SARI, using CXR-based score of 4 or more, TLC of less than 8,700 cells/μL, and NLR of

β-Thalassaemia, the most common monogenic disorder, is characterized by genetic heterogeneity at the molecular level. More than 300 mutations of the β globin gene have been characterized all over the world, however, few common mutations account for majority of the cases in various populations. The present study aimed to screen known cases of β-thalassaemia in the Western part of Rajasthan state for five common mutations. The study included 144 known cases of β-thalassaemia of all clinical phenotypes. Cases were diagnosed based on clinical features, haematology investigations including haemogram and Hb-HPLC. Blood samples from cases were taken for mutation analysis. After DNA extraction, mutations were characterized by the polymerase chain reaction method employing allele specific priming technique (AMRS) to study the five mutations including IVS-I-5 (G → C), IVS-I-1 (G → T), CD41/42 (–TCTT), CD 8/9 (+G) and 619 bp deletion from the 3′ end of the β-globin gene using a total of seven different primers. Of all 144 cases, 74 (51.38% of all) cases were of β-thalassaemia major, five (3.4% of all) cases were of β-thalassaemia intermedia and 65 (45.14% of all) cases were of β-thalassaemia minor. Mutation analysis revealed that five common mutations were present in 130 (90.27% of all) cases. Among identified mutations, highest frequency of mutation was of IVS-I-5 (G → C) identified in 73 cases (50.7% of all cases). In 11 (7.63% of all) cases, more than one mutation was identified. β-Thalassaemias are common in Western Rajasthan; however, there is dearth of literature from this part of the country. We observed that five common mutations are common in this part of the country also. These observations are helping us in forming the basis for comprehensive diagnostic database that would not only be useful for genetic counselling but also for prenatal diagnosis.

Context: The arid climate of Western Rajasthan is challenging for malaria transmission, with the number of cases correlating directly with the annual rainfall pattern. Moreover, >90% of the cases in this region are caused by Plasmodium vivax, which has recently been shown to cause a similar degree of thrombocytopenia as Plasmodium falciparum. Aims and Objectives: The aim of the study was to determine the degree of thrombocytopenia in malaria patients and its association with different species of malaria in this region with an unstable malaria epidemiology. Materials and Methods: This retrospective study was conducted on all microbiologically confirmed malaria patients with documented platelet counts from August 2017 to October 2018. Microbiological diagnosis was established by rapid diagnostic tests and peripheral blood film examination. Platelet counts were used to assess the degree of thrombocytopenia. Results: A total of 130 cases were included in the study, of which 118 (91%) were caused by P. vivax, while the rest 12 (9%) were caused by P. falciparum. Thrombocytopenia was present in 108 (83%) cases, and the mean values of platelets in thrombocytopenic patients with P. vivax and P. falciparum infection were 72600/μL and 48500/μL, respectively. Although P. falciparum infection was significantly associated with severe thrombocytopenia (odds ratio: 4.7, [95% confidence interval 1.3–16.1]), extremely low platelet counts (n = 5) warranting platelet transfusions (n = 1) were seen only in P. vivax cases. Only one patient required platelet transfusions in these patients suggesting good tolerance to thrombocytopenia. Conclusions: Avoiding unnecessary transfusions in febrile thrombocytopenic patients with an established malaria diagnosis can help in reducing transfusion-transmitted infections.

BackgroundAccurate diagnosis of anemia by community workers using a point-of-care device is a challenge. The objective of the study was to establish the diagnostic accuracy of point-of-care devices for detecting anemia in community settings.MethodsIt was diagnostic accuracy study with cross-sectional design on adult patients attending the outpatient department of rural/ urban health centres of Medical colleges from India. The index tests were HemoCue, TrueHb, Massimo’s device and spectroscopic device, compared against autoanalyzer (gold standard). Accuracy was expressed by sensitivity, specificity, likelihood ratios, predictive values, area under the curve (AUC) and levels of agreement. For the diagnostic accuracy component, 1407 participants were recruited with a minimum of 600 for each device. An additional 200 participants were considered to elucidate the performance of devices in different weather conditions.ResultsHemoCue and TrueHb performed better than Massimo and spectroscopic devices. Detection of anemia by technicians was similar between TrueHb and HemoCue (AUC 0.92 v/s 0.90,p > 0.05). Community workers performed better with Hemocue for detecting anemia compared to TrueHb (AUC 0.92 v/s 0.90,p p p HemoCue showed a bias or mean difference (95%CI) of 0.47 g/dl (0.42, 0.52) for all values, and 0.92 g/dl (0.82, 1.03) for severe anemia. For TrueHb, it was − 0.28 g/dl (− 0.37, − 0.20) for all readings, and 0.06 g/dl (− 0.52, 0.63) for severe anemia. TrueHb appeared to be more consistent across different weather conditions, although it overestimated Hb in extreme cold weather conditions.ConclusionFor detection of anemia, True Hb and HemoCue were comparable. For severe anemia, True Hb seemed to be a better and feasible point-of-care device for detecting anemia in the community settings.

Progressive disseminated histoplasmosis (PDH) usually presents as fever, anemia, leukopenia, hepatosplenomegaly, lymphadenopathy and pulmonary symptoms. There are few reports showed the association of Idiopathic CD4 lymphocytopenia (ICL) with histoplasmosis. We describe a 65-year-old female presented with history of fever, papulo-nodular rash and significant weight loss and diagnosed as progressive disseminated histoplasmosis. All immunocompromised conditions were ruled out. In addition, her 2 consecutive CD4 counts were below 300. The patient was diagnosed with PDH associated with ICL. Patient showed significant improvement with liposomal amphotericin B and itraconazole. Absolute CD4 counts should be done in all cases of progressive disseminated histoplasmosis even in HIV negative individuals to rule out associated ICL.

Background:: The understanding of pathogenesis is necessary for the development of effective treatment for COVID-19. Various studies have postulated that there is a complex interplay of mediators of coagulation and inflammation responsible for the pathogenesis of COVID-19. We did this study on coagulation parameters and inflammatory markers and their effect on outcome in patients with COVID-19. Methods: This was a single centre observational cross-sectional study. Procoagulants [Prothrombin time (PT), activated partial thromboplastin time (APTT), D-dimer, lupus anticoagulant (LA), fibrinogen, factor-VIII (F-VIII)]; anticoagulants [protein-C (PC), protein-S (PS), antithrombin] and inflammatory markers [interleukin-6 (IL-6) and highly sensitive – C-reactive protein (hs-CRP)] were measured at the time of hospitalization and correlated with the severity of the disease. Results: A total of 230 patients were enrolled, of which 61.3%, 20.0%, and 18.7% had asymptomatic/ mild, moderate, or severe disease, respectively. COVID-19 disease severity was associated with rising trends with coagulation parameters (PT, APTT, D-Dimer; p value 0.01, Conclusions: This study revealed increased levels of coagulation and inflammatory parameters, which correlated with the severity of COVID-19. Age, D-dimer, IL-6, hs-CRP, APTT, fibrinogen, and Factor VIII were significantly higher in patients with moderate and severe disease as compared to asymptomatic/mild disease. Advanced age, high D-dimer, and high hs-CRP were significantly associated with poor outcomes.

The use of aromatherapy for the reduction of anxiety levels during dental treatment procedures has been well established in the literature; however, there is limited evidence regarding its efficacy. The present meta-analysis is an attempt to assess the association between the use of aromatherapy and anxiety levels among dental patients. A comprehensive search was conducted across Medline, Scopus, Web of Science, EBSCO host, Cochrane databases, and Google Scholar for studies evaluating aromatherapy and anxiety level among dental patients. PRISMA guidelines were followed for the meta-analysis. Randomized and cluster-randomized trials comparing aromatherapy with controls were included. The random-effects model was used to assess the mean differences in anxiety levels of patients visiting dental OPD. The significance value was set at P < 0.05. Six studies were identified that met the requirements for inclusion. Aromatherapy was significantly associated with reduction in patient anxiety levels during dental treatment (pooled mean difference: -3.36 [95% CI, -3.77 - -2.95, P = 0.00001). Low heterogeneity was noted between studies (I2 = 1%, P = 0.41) analyzed in the meta-analysis. High certainty of the evidence was obtained from the association between the use of aromatherapy and dental anxiety. This meta-analysis suggests that aromatherapy is effective in reducing dental anxiety. When used judiciously, the results of this work should encourage the use of aromatherapy to reduce patient anxiety levels during dental procedures.

Finding spurious thrombocytopenia is a common occurrence in clinical practice, whereas pseudothrombocytosis is a very uncommon event. Despite several technical advancements in automated haematology analysers, a careful peripheral smear examination remains standard examination in cases of discrepancy of platelet counts. We are presenting a case of betathalassaemia intermedia who had pseudothrombocytosis, which was falsely labelled as myeloproliferative neoplasm before the patient visited our haematology clinic.

Tuberculosis (TB) is an important public health problem in developing countries. In India, despite substantial efforts targeting TB and its associated risk factors, the number of cases remains high with 2.7 million new cases per year with a minimum 10% case contributed by paediatric TB. Disseminated TB has been increasingly recognised in children in recent times due to the increased prevalence of immune suppression secondary to AIDS and immunosuppressive therapies for various medical disorders and increasing awareness. Here we describe a 5-year-old girl who presented with fever of unknown cause, and her diagnosis of disseminated TB was delayed due to atypical presentation and the paucibacillary nature of paediatric TB. It was a bone marrow examination report which led us to clinch the diagnosis. The case highlights the difficulty in diagnosing disseminated TB and the importance of bone marrow examination in such cases.

Chronic myeloid leukemia (CML) is predominantly a disease of adults and the elderly. CML is uncommon in the paediatric age. Priapism as an initial presentation is quite uncommon in CML and is rare in the case of CML in paediatric and adolescent age. We present a case of CML in an adolescent male who presented with priapism of 48 hours duration. The patient was managed in an emergency by corporal aspiration and saline irrigation and was found to have CML during a hospital stay. The patient was treated with imatinib and is under follow-up for the last two years.

Creutzfeldt–Jakob disease (CJD) is a well-described disease. It is characterized by rapidly progressive dementia, myoclonus, ataxia, pyramidal, and extrapyramidal signs. There are well-defined electroencephalogram and magnetic resonance imaging (MRI) findings, and markers found in the cerebrospinal fluid (CSF). The gold standard for diagnosing CJD remains brain biopsy. We present a case of a patient with a family history of biopsy-proven CJD who initially presented with symptoms of peripheral neuropathy. A month later, he developed ataxia, ophthalmoparesis, and then dysarthria. His initial workup was relatively unrevealing, showing an elevated protein in his CSF. He was thought to have Miller Fisher syndrome variant of Guillain–Barré syndrome. He neither, however, responded to plasmapheresis nor IVIG. He later started to develop progressive dementia. Repeated MRI showed restricted diffusion in the caudate and putamen, as well as in the cortex (cortical ribboning). Lumbar puncture was then found to be positive for 14-3-3 protein, total-tau protein, and real-time quaking-induced conversion assay, which are highly suggestive of CJD. We present a case of CJD with an unusual presentation resulting in misdiagnosis, prolonged workup, and potentially harmful treatment modalities. This case highlights the importance of broadening our definition of CJD to encompass more cases with unusual presentations.

Antiphospholipid syndrome (APS), an autoantibody mediated disease, is characterized by presence of antibodies against the proteins bound to the phospholipid membranes. The antibodies are predominantly formed against beta-2-glycoprotein I (b2GPI) which is considered pathogenic, but presence of lupus anticoagulant is a predictor of thrombotic events. The thrombotic events in APS may manifest as venous or arterial or small vessel thrombosis in any tissue or organ and pregnancy related complications namely, recurrent (three or more) and early spontaneous miscarriages before 10 weeks of gestation or unexplained deaths of normal fetus at or beyond 10 weeks, eclampsia or severe pre-eclampsia, intra-uterine growth retardation and pre-term births. However, lately its role as an etiological mechanism in causation of certain neuro-psychiatric disorders has been put forth. It has been suggested that one should suspect APS in psychiatric manifestations which are atypical, resistant to treatment, associated with cognitive decline and dementia, abnormal involuntary movements, livedo reticularis, migraine, thrombotic events like stroke or transient ischemic attacks, obstetrical complications. In this brief communication, we describe the case of young male who has been suffering from treatment resistant and difficult to manage bipolar affective disorder (BPAD) with fluctuating thrombocytopenia and neurological findings with positive lupus anticoagulant. We propose it to be a consequence of an atypical presentation of APS.

We report the case of an 84-year-old female presented to us with acute onset altered sensorium. On investigation, neurological and infectious causes were ruled out. On further evaluation, her serum calcium was found elevated (15.07 gm/dl). The diagnosis of hypercalcemic encephalopathy was made with the possibility of multiple myeloma due to raised total protein and globulin levels. Serum electrophoresis, immunofixation, and bone marrow examination confirmed the diagnosis of multiple myeloma. The patient was treated with bortezomib, dexamethasone, and lenalidomide. After 1 week, she improved with normalization of serum calcium. Herein, we highlight hypercalcemia as an important cause of encephalopathy. As our report suggests, metabolic encephalopathy can be the first presentation in multiple myeloma.

Objective Using video-EEG (v-EEG) diagnosis as a gold standard, we assessed the predictive diagnostic value of home videos of spells with or without additional limited demographic data in US veterans referred for evaluation of epilepsy. Veterans, in particular, stand to benefit from improved diagnostic tools given higher rates of PNES and limited accessibility to care. Methods This was a prospective, blinded diagnostic accuracy study in adults conducted at the Houston VA Medical Center from 12/2015–06/2019. Patients with a definitive diagnosis of epileptic seizures (ES), psychogenic nonepileptic seizures (PNES), or physiologic nonepileptic events (PhysNEE) from v-EEG monitoring were asked to submit home videos. Four board-certified epileptologists blinded to the original diagnosis formulated a diagnostic impression based upon the home video review alone and video plus limited demographic data. Results Fifty patients (30 males; mean age 47.7 years) submitted home videos. Of these, 14 had ES, 33 had PNES, and three had PhysNEE diagnosed by v-EEG. The diagnostic accuracy by video alone was 88.0%, with a sensitivity of 83.9% and specificity of 89.6%. Providing raters with basic patient demographic information in addition to the home videos did not significantly improve diagnostic accuracy when comparing to reviewing the videos alone. Inter-rater agreement between four raters based on video was moderate with both videos alone (kappa = 0.59) and video plus limited demographic data (kappa = 0.60). Significance This study demonstrated that home videos of paroxysmal events could be an important tool in reliably diagnosing ES vs. PNES in veterans referred for evaluation of epilepsy when interpreted by experts. A moderate inter-rater reliability was observed in this study.

Bone marrow microenvironment plays a crucial role in the growth of hemopoietic cells and bone marrow function, which in turn depends on an intact microvasculature. Our study assesses the microvessel density (MVD) in the bone marrow of aplastic anemia (AA) patients, compares with MVD of controls and MVD among the different types of AA. Bone marrow specimens from 60 patients with AA and 17 controls were studied. There were 33 patients with non severe AA (NSAA), 12 patients with severe AA (SAA) and 15 patients with very severe AA (VSAA). MVD was calculated on sections stained immunohistochemically for CD34. The mean bone marrow MVD in AA group was 1.28 ± 0.36, being significantly lower than that in control group (6.80 ± 1.59, p

Brucellosis is a common zoonotic disease worldwide. It has protean clinical manifestation and sometimes may has a life-threatening complication. A 4-year-old boy presented with a history of fever, myalgia and appetite loss for 3 weeks. On examination, he had hepatosplenomegaly. The initial working diagnosis was an infection, autoimmune disease and malignancy. Investigations showed positive Brucella serology, and he was started on rifampicin and cotrimoxazole. He was further investigated because of persistent fever, which revealed evidence of haemophagocytic lymphohistiocytosis (HLH). He continued treatment for brucellosis, except rifampicin which was replaced with doxycyclin due to a worsening liver function. The child showed complete clinical and biochemical improvement after 6 weeks of therapy. HLH is a life-threatening condition and should be suspected in children with brucellosis, who did not respond to appropriate antibiotics treatment. Secondary HLH does not always require specific therapy; it may improve with adequate treatment of the underlying condition.

Recurrent hemarthrosis is a common entity in children. Although recurrent hemarthrosis most often associated with hemophilia (VIII or IX deficiency), but rarely it can be associated with factor VII deficiency (FVIID). It is a strong mimicker of hemophilic hemarthrosis. Once hemophilia is excluded as a cause of recurrent hemarthrosis, congenital FVIID needs to be considered for long-term planning of treatment and avoiding unnecessary transfusion of factor concentrates. Clinical presentation of FVIID has a varied spectrum and does not correlate with factor levels. Here, we present a case of recurrent hemarthrosis secondary to FVIID.

Background: Neonatal jaundice is the most commonly observed clinical condition of early neonatal period. It remains a common cause of readmission and important cause of concern for the parents and clinicians. Therefore, appropriate management of neonatal hyperbilirubinemia is of paramount importance. Phototherapy plays a significant role in its management but this modality is not devoid of complications. Authors studied electrolyte changes in the neonates, who received phototherapy.Methods: This perspective hospital based observational study conducted over the period of one year on 90 eligible admitted neonates who received phototherapy as per AAP guideline. Serum bilirubin, sodium, potassium and calcium were determined before and after termination of phototherapy. The first samples were considered as controls and compared with the samples collected after termination of phototherapy.Results: The incidence of LBW babies in this study was 21.9%, while mean birth weight and gestational age was2.76±0.38 kg and 38.34±0.88 weeks respectively. Mean duration of phototherapy was 38.48±09.34 hours. The mean sodium, potassium and calcium level before therapy were 146.6±6.2 mg/dl, 4.7±0.47 mg/dl and 9.4±0.73 mg/dl respectively. Authors found statistically significant changes in the electrolyte’s levels (Na+ 141.3±6.1, K+ 4.2±0.51 and Ca+2 8.4±0.68 respectively), after phototherapy however none of any neonate shown any clinical signs of dyselectolytemia.Conclusions: Neonates undergoing phototherapy are at greater risk of dyselectrolytemia especially in preterm babies therefore close clinical and biochemical monitoring is required for the prompt management of any significant electrolyte imbalance.

We report a case of 56-year-old man presented to us with chief complaints of frothy urine and leg swelling. A urinalysis revealed nephrotic-range proteinuria. Haematological investigations revealed thrombocytosis, leucocytosis and peripheral blood smear showed a leucoerythroblastic picture. JAK 2 mutation was positive. To confirm the diagnosis of myeloproliferative neoplasm, bone marrow biopsy was done, which was suggestive of primary myelofibrosis. The patient underwent kidney biopsy due to rapidly declining renal function and persistent proteinuria, which was suggestive of focal segmental glomerulosclerosis. Early glomerulopathy is rare in myeloproliferative neoplasm, and aggressive follow-up is required to prevent progression of kidney disease.

Objectives Myeloperoxidase (MPO) detection either by enzyme cytochemistry (cMPO) or flow cytometry (fMPO) plays a major role in acute leukaemia (AL) diagnosis as per World Health Organization (WHO) 2008 classification. Although 3% cMPO was recommended as positivity, no specific cut-off had been mentioned by WHO for fMPO. Various authors recommend different cut-offs ranging from 3 to 28% for fMPO. The aim of this study was to analyse fMPO cut-offs ranging from 3 to 10% in classifying AL and to assess whether a new cut-off could be suggested. Methods Totally, 216 cases of AL were retrospectively analysed for fMPO ranging from 3 to 10% and compared with gold standard. Presence of cMPO (≥3%) and/or expression of two or more pan-myeloid markers (CD13, CD33, and CD117) in the absence of CD19 and CD3 were kept as gold standard for diagnosis of acute myeloid leukaemia (AML). Results Sensitivities for classifying AL as AML/mixed phenotypic acute leukaemia (MPAL) at 3, 5.4, and 10% were 98.3, 98.3, and 96.6%, respectively, whereas specificities at this cut-off were 22.2, 91, and 71%, respectively. Discussion Only few studies have been done in this aspect to define a consistent cut-off for fMPO for proper classification of acute leukaemias. This was one of the largest and few studies available till date in this regard. Conclusion The newer cut-off for fMPO (5.4%) emerged out from our study with best sensitivity and specificity for accurately classifying AL cases into acute lymphoblastic leukaemia, AML, and MPAL.

Aplastic anemia (AA) is a life-threatening bone marrow failure disorder, if untreated, is associated with very high mortality. Allogenic bone marrow transplantation (BMT) is the standard of care for severe aplastic anemia (SAA) patients those who are younger than 40 years of age. The development of secondary malignancies in post-BMT setting for AA is a rare, however, well documented phenomenon. Among the secondary malignancies, development of acute myeloid leukemia is even rarer entity. Here we report a case of acute myeloid leukemia following human leucocyte antigen (HLA) matched sibling peripheral blood stem cell transplant (PBSCT) in a case of SAA. The patient achieved complete remission (CR) following chemotherapy and in CR1, a second HLA matched PBSCT from a different donor was offered. The patient is presently in remission at day +180 post-PBSCT.

Introduction: Hairy cell leukemia is a rare chronic B-cell disorder that follows an indolent but progressive course. This disorder is characterized by pancytopenia, splenomegaly, bone marrow fibrosis and the presence of atypical lymphoid cells with hairy projections in peripheral blood, bone marrow and spleen. Treatment is mainly with nucleoside analog cladribine, which induces complete remission in up to 85% cases. Materials and Methods: This is a retrospective analysis of Hairy cell Leukemia cases diagnosed and treated in the Department of Hematology, All India Institute of Medical Sciences, New Delhi between 2002 and 2013. Various parameters such as clinical features, laboratory parameters including complete blood cell count, bone marrow findings, cytochemistry, immunophenotyping by flowcytometry or immunohistochemistry, treatment protocol and complications secondary to treatment and relapse were reviewed. Results: A total of 35 cases were diagnosed during this period of 12 years of which 27 received cladribine and went in to remission. Median follow-up duration was 26 months. 5 (18%) cases had a relapse and all relapsed cases achieved second remission with cladribine; however, there was no case of second malignancy in our cohort. Conclusion: Cladribine has emerged as the treatment of choice for hairy cell leukemia given that the overwhelming majority of patients achieve long-lasting complete remissions. Upon relapse, these patients could be successfully salvaged with cladribine retreatment.

Bone marrow fibrosis has been associated with different types of non-neoplastic conditions like granulomatous and autoimmune diseases and a variety of neoplastic disorders such as acute megakaryoblastic leukemia, Hodgkin lymphoma, non-Hodgkin lymphoma and myeloproliferative neoplsms. Therapy induced fibrosis is a rare phenomenon. Here we report a case of an incidentally diagnosed acute promyelocytic leukemia (APL) with t(11;17) which was treated with arsenic trioxide (ATO) for 45 days. However, the patient did not go into remission and developed massive fibrosis of bone marrow. Literature search does not reveal such documented marrow fibrosis following therapy with ATO in a case of APL., Highlights • During pre-op evaluation for hysterectomy in a 44 year old, a high TLC was noted. • APL with 45X,−X, t(11;17)(q23;q21) was diagnosed on bone marrow examination. • Treated with ATO, day+45 bone marrow biopsy revealed diffuse fibrosis. • Case is reported for this unusual feature of ATO-induced bone marrow fibrosis.

Transfusion-acquired hemoglobinopathy occurs when a carrier of hemoglobinopathy with no significant abnormalities donates blood, and the blood is transfused to a recipient. This process can lead to spurious results in the recipient without any clinical abnormality or infrequently can result in disastrous situations. The incidental finding of such posttransfusion related abnormal peaks in hemoglobin high-performance liquid chromatography (Hb HPLC) may cause diagnostic dilemmas and result in unnecessary laboratory testing. Here, we report two such cases of transfusion-acquired hemoglobinopathies, which were subsequently resolved by the abnormally low percentage of the Hb variants, transient nature of the peaks, and parental Hb HPLC.

Dear editor, Priapism, that is, persistent penile erection that continues hours beyond, or is unrelated to, sexual stimulation, is a relatively uncommon disorder and is a medical emergency. Typically, only the corpora cavernosa are affected [1]. The term priapism was derived from the Greek god Priapus, son of Aphrodite who was born with oversized genitals [2]. The haematological causes for priapism include Sickle cell anaemia, Leukaemia, Multiple myeloma, Paroxysmal nocturnal haemoglobinuria, Thalassaemia, Thrombocythemia and Henoch–Schonlein purpura [3]. As far as hemoglobinopathies are concerned, priapism is most often encountered in sickle cell disease in 38–42 % of cases followed by sickle/beta thalassemia [4, 5]. Priapism is also noted in patients with sickle cell trait even though the incidence is low as compared with sickle cell anaemia [6–9]. However, so far priapism in Hb E homozygosity is not reported in literature. In this correspondence we intend to bring to notice an unusual association of priapism with Hb E homozygosity. A 27 years old male, resident of North Eastern part of India was referred to our haematology OPD to rule out any underlying haematological disorder as the individual had an episode of priapism 3 months ago. The patient had an episode of unprovoked painful persistent erection for more than 8 h for which he sought medical advice. The surgeon at that medical center had managed with therapeutic needle aspiration from corpora cavernosa combined with flushing of cavernosa with normal saline to clear the sludged blood. The patient’s symptom subsided subsequently with the intervention and was referred to our center for further evaluation. On detailed clinical history, the individual was not on any medication for chronic illnesses; neither was he abusing any psychoactive drugs, alcohol, no history of prior trauma to the perineal region. His physical examination revealed mild pallor. There was no hepatosplenomegaly or lymphadenopathy. Systemic examination did not reveal any abnormality. On USG abdomen, spleen was not reported as enlarged (Span 13 cms). On investigations, haemoglobin, total leucocyte count and platelet count were 9.1 gm/dL, 7.1 9 10/lL and 118 9 10/lL respectively. Peripheral smear examination revealed microcytic hypochromic red cells and target cells with normal differential leucocyte count and reticulocyte count of one percent. There were no sickle cells or atypical cells in peripheral smear and sickling test was also negative. Subsequently Hb HPLC was performed which revealed with Hb A2 ? E of 92.6 % (Retention time3.68 min), Hb A of 6 % (Retention time 2.29 min) and Hb F (Retention time 1.06 min) of 1.6 % (Fig. 1; Table 1). This was followed by parental study which revealed Hb A2 ? E in mother and father of 29 and 27 % respectively suggestive of both parents being heterozygous for Hb E, S. Venkatesan A. Purohit (&) M. Aggarwal P. K. Singh T. Seth H. P. Pati Department of Hematology, All India Institute of Medical Sciences, New Delhi, India e-mail: purohitabhi80@gmail.com

Background: Bleeding is a common clinical presentation. Even patients with mild bleeding disorders are extensively investigated for ascertaining the cause. The present study was conducted in order to evaluate the extent of the possibility of diagnosis in mild bleeding disorders. Material and Methods: This was a prospective study of patients referred for work up of mild bleeding for a period of 13 months. A complete blood count, peripheral smear examination, Prothrombin time, Partial Thromboplastin time and Thrombin Time, Platelet Aggregometry test, tests for von Willebrand’s disease and Platelet function 3 availability were measured. Results: 164 patients presented with mild bleeding, in 114 of the patients a single site of bleeding was present. Epistaxis was the most common presentation (39%). Cutaneous bleeding (petechiae and purpura) was the next common site. History of a major bleeding tendency in the family was present only in 11 patients. The investigations showed that VWD (17/164), followed by clotting disorders (CD) mainly mild hemophilia (15/164) were the most common diagnosable cause. There were also 4 cases of hypofibrinogenemia. The disorders of platelets (Platelet function defects/PFD) were the least common (9/164). Rest 123 (75%) patients could not be diagnosed on the basis of these investigations and were labeled as Bleeding disorders – Unclassified (BDC). Conclusion: n our study, 75% of the patients with mild bleeding remained undiagnosed even after extensive laboratory workup, thus raising a very pertinent question that is it necessary that all mild bleeders submit to a broad battery of investigations, as the diagnosis continues to be elusive despite extensive workup.

b>Introduction and Materials and Methods: Early diagnosis of sepsis is extremely important to reduce high mortality and morbidity. In this study, clinical usefulness of the volume, conductivity and scatter parameters (mean channels of cell volume, conductivity, and light scatter) in neutrophils was analyzed for predicting acute bacterial infection, which are obtained by the Coulter LH 750 Hematology Analyzer (Beckman Coulter, Fullerton, CA, USA) during automated differential counts. Results: Peripheral blood samples from 162 patients with positive blood cultures for bacteria and 40 healthy controls were studied. We observed a significant increase in the mean channel of neutrophil volume (MNV) from septic patients compared with control subjects (156 ± 13.5 vs. 143 ± 4.8; P < 001). Discussion and Conclusion: An elevation of the MNV was associated with a higher white blood cell count and percentage of neutrophils and was present even in patients who did not have leukocytosis or neutrophilia. With a cut-off of 149 for the MNV, a specificity of 91.4% and sensitivity of 88.7% were achieved. As a quantitative, objective, and more sensitive parameter, we propose that the MNV has a potential to be an additional indicator for acute bacterial infection.

Diagnosis of immune thrombocytopenia (ITP) is based on clinical suspicion and normal peripheral smear except for thrombocytopenia. Bone marrow examination is carried out to rule out leukemia, myelodysplastic syndrome or aplastic anemia. However, in most cases, clinical diagnosis is not altered after the bone marrow reports. Hence, this present study was carried out to evaluate the justification for bone marrow examination in the setting of isolated thrombocytopenia. All patients presenting to the hematology OPD with isolated thrombocytopenia and suspected diagnosis of ITP, between October 2011 and April 2013, were included in the study. Data was collected from bone marrow reports and outpatient records. A total of 353 cases were found. 319 cases had features of typical ITP and the rest had some form of organomegaly and/or lymphadenopathy. Bone marrow examination in all cases revealed normal hematopoietic elements and prominence of megakaryocytes including juvenile forms with no novel diagnosis in any patient. Routine use of bone marrow examination in the diagnostic workup of isolated thrombocytopenia is not required in our center even if steroids are planned as a first line therapy. However, a detailed history, thorough examination with complete hemogram and peripheral smear examination are essential.

T lymphoblastic leukaemia (T-ALL) is a neoplasm of lymphoblasts committed to T-cell lineage and T-ALL is diagnosed by identification of immaturity markers and the lineage specific Cluster of Differentiation (CD) marker, CD3 in the leukemic cells. Here we report a 20 year old male who was diagnosed as case of T-ALL, whose leukemic blasts on morphology resembled the lymphoid cells seen in hairy cell leukemia (HCL) resulting in a diagnostic dilemma which was resolved subsequently by immunophenotyping. Even though the presence of cells with hairy projections is rarely reported in haematological malignancies other than HCL, ours is a first of its kind in TALL.

Dear Editor, Hairy cell leukemia (HCL) is an uncommon but distinct form of chronic B cell lymphoproliferative disorder, comprising about two percent of lymphoid leukemias. The pathologic diagnosis of HCL is based mainly on morphology and immunophenotype leukemic cells. HCL has a characteristic immunophenotypic profile and light scatter characteristics. The tumor cells express B cell–associated markers, CD19, CD20, CD22, and CD79b, and are characteristically positive for CD103, CD11c, and CD25 and usually negative for CD10, CD5, and CD23 [1]. However, atypical immunophenotypes have been reported in otherwise typical HCL [2–5]. We report a case of HCL which had usual clinical and morphological findings, however expressed several unusual aberrant immunophenotypic markers. A 32 year old male presented with 2 month history of weakness and awareness of mass in left upper side of abdomen. On examination, he had pallor and palpable spleen 20 cms below left costal margin, however, there was no icterus, lymphadenopathy or hepatomegaly. Other systemic examination was within normal limits. Laboratory parameters revealed haemoglobin, total leucocyte count and platelet count of 10.7 gm/dl, 27 × 103 and 45 × 103/cumm respectively. Peripheral smear examination revealed 72 % of mononuclear cells with abundant cytoplasm and circumferential hairy projections which were positive for TRAP staining. Flowcytometric immunophenotyping (Fig. 1) revealed features characteristic of HCL, that is, positivity for CD19, CD11C, CD25 and CD103. In addition, these CD19 positive cells showed co-expression of CD5, CD 10 and CD 2 while negative for CD3 and CD23. Further these cells were positive for CD20, CD200, FMC7 along with lambda light chain restriction. Subsequent bone marrow examination revealed infiltration by hairy cells with characteristic ‘fried egg’ appearance in bone marrow biopsy. Fig. 1 Flow cytometry dot plots showing that the neoplastic B-cells co-express CD11c, CD25, CD103 with lambda light chain restriction. The cells also express FMC7,CD2, CD5 and CD 10 With final diagnosis of HCL, patient was treated with cladribine @ 0.09 mg/kg for 7 days with prophylactic cotrimoxazole, fluconazole, and aciclovir from admission till discharge. The patient tolerated chemotherapy well without infusional reactions, however was started on G- CSF from day 9 of therapy. His spleen size regressed to two cms below costal margin. Patient is presently in complete hematological remission (CR) for past 3 months. Aberrant expression of T-cell–associated antigens on B cell non-Hodgkin lymphomas (NHLs) other than CD43 is a known but uncommonly observed phenomenon. Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) is the most cited B-cell malignant neoplasm with aberrant coexpression of T-cell–associated antigens [6]. Hairy cell leukemia (HCL) exhibits a characteristic immunophenotypic profile that is strongly positive for pan–B-cell markers and positivity for CD103, CD11c, and CD25; and usually negative for CD5, CD10, and CD2. The present case revealed aberrant expression of all these three markers which is extremely unusual in HCL. Chen et al. [7] evaluated 35 HCL cases and identified CD10 expression which is a marker for B-cell neoplasms of follicular center origin in five (14 %) cases. The frequencies of CD10 expression in HCL in the reported studies ranged from 5 to 26 % [5]. CD2 and CD5 have been shown to be rarely expressed in HCL cases. Of 169 cases of typical HCL evaluated by Shao H et al. three cases (2 %) showed expression of CD2 and four cases (2 %) showed expression of CD5 respectively. However the expression of CD 2 is more often seen in HCL variant cases to the tune of nine percent. Occurrence of a second concurrent monoclonal B cell population is mentioned in the same study with the second clone exhibiting a CLL/non specific IPT features. The light chain expression between the two monoclonal B-cell populations was different in all three patients, indicating different clonal origins of the two populations [8]. However in our case it was a single clonal population ruling out a second clone which is responsible for expression of CD2, CD5 and CD10. As far as minimal residual disease (MRD) detection in HCL is concerned flowcytometry is more sensitive and more specific and it also permits quantitation of tumor cell number [9]. Hence, the valuable information of this aberrant leukemia associated immunophenotype (LAIP) expressed in HCL cases adds more specificity in MRD analysis. In a resource limited setting, application of minimal antibody panel for diagnosis of lymphoma/leukemia by flowcytometry would result in non identification and under reporting of valuable information suchas aberrant antigen expression. This information will not only aid in correct diagnosis of cases but also will aid during follow up in the form of MRD analysis. Also use of a limited antibody panel may result in misdiagnosis in select cases, due to aberrant antigen expression. Hence this communication indicates that it is not uncommon for HCL to display an unusual immunophenotype. Recognition of the variability of immunophenotype and correlating with morphologic and clinical features are essential for establishing an accurate diagnosis of HCL. The distinction between HCL and other chronic B-cell lymphoproliferative disorders is clinically important because patients with HCL do not respond well to conventional lymphoma chemotherapy but are highly sensitive to purine analogues such as cladribine (2-chlorodeoxyadenosine) and pentostatin (2′-deoxycoformycin). Further, even though the HCL may show immunophenotypic aberrations, the outcome of these cases are comparable with those exhibiting the usual IPT features as brought by Chen et al. [7]. The present case also responded well and is in remission for the past 3 months following therapy with cladribine. The Manuscript has been approved by the Institute’s ethics committee and has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments. A written informed consent was obtained from the patient prior to the procedures and treatment. Details that might disclose the identity of the subjects under study have been omitted.

Precursor Natural Killer (NK) cell lymphoblastic leukemia/lymphoma is a rare entity defined clearly by WHO (2008 WHO classification). However, the pathobiology of this subset of neoplasms is not clearly defined. There is wide disparity in the literature regarding the nomenclature and diagnostic criteria used to diagnose and characterize acute leukemias of presumed NK cell origin. In the present article we report a case of Precursor NK cell lymphoblastic leukemia/lymphoma and review the cases reported after 2008 WHO classification came into vogue, as acute leukemias of NK cell origin.

A middle aged male presented to us with an unusual problem when his acute lymphoblastic leukemia (ALL) disappeared without any chemotherapy. We faced a dilemma whether to go ahead and treat his initial diagnosis or wait. Eventually he did relapse and was treated, albeit with a fatal outcome. Such spontaneous remission in acute leukemia are a very rare event, more common in acute myeloid leukemia and in children. Spontaneous remission in adult ALL is rarely described in literature.

Systemic mastocytosis is a rare clonal disorder characterized by mast cell infiltration of one or more organs, with or without skin involvement. Leonine facies is a rare presentation and corresponds to the morphologic manifestation of diffuse dermal infiltration of the face as a result of long standing disease. Bone marrow aspiration and biopsy findings in a 60-year-old woman, who had extensive cutaneous infiltration due to systemic mastocytosis, resulting in 'leonine facies,' are described, and causes of leonine facies are discussed.

Bone marrow necrosis (BMN) is seen in various malignant and non-malignant disorders. The relative frequency ranges between 0.37 and 6.5 % in western countries. The aim of this study is to analyze various etiology and prevalence at our tertiary care center and to compare our clinico-pathologic profile of this uncommon entity to western data. This is a retrospective observational study done in the Department of Hematology, tertiary care centre in northern India over a period of 21 months. Various clinical features and laboratory parameters of the cases showing BMN were noted from hospital records. Bone marrow trephine biopsies with BMN were graded semi-quantitatively according to the criteria laid down by Maisel et al. grading system into three grades I, II, and III as area of the biopsy showing necrosis like 50 %, respectively. Bone marrow necrosis was observed in 1.8 % of bone marrow biopsies studied for various clinical indications over the study period. Median age was 35 years with male to female ratio of 3.4:1. Underlying non-malignant disease was seen in 64.4 % cases with tuberculosis as the most common etiology (59.32 %). Among malignancies, hemato-lymphoid malignancies were observed in 23.72 % cases. On semi-quantitative grading, bone marrow biopsies revealed grade I, II, and III necrosis in 62.71, 18.64, and 18.64 % of cases, respectively. BMN is rare clinico-pathological entity with a poor prognosis. Although the etiology of BMN is varied, this differential possibility should be kept in mind in any patient presenting with bicytopenia or pancytopenia especially in the setting of infection and malignancy. Tuberculosis should always be an important differential diagnosis in developing countries like India, before considering malignancy.

Diagnosis of immune thrombocytopenia (ITP) is based on clinical suspicion and normal peripheral smear except for thrombocytopenia. Bone marrow examination is carried out to rule out leukemia, myelodysplastic syndrome or aplastic anemia. However, in most cases, clinical diagnosis is not altered after the bone marrow reports. Hence, this present study was carried out to evaluate the justification for bone marrow examination in the setting of isolated thrombocytopenia. All patients presenting to the hematology OPD with isolated thrombocytopenia and suspected diagnosis of ITP, between October 2011 and April 2013, were included in the study. Data was collected from bone marrow reports and outpatient records. A total of 353 cases were found. 319 cases had features of typical ITP and the rest had some form of organomegaly and/or lymphadenopathy. Bone marrow examination in all cases revealed normal hematopoietic elements and prominence of megakaryocytes including juvenile forms with no novel diagnosis in any patient. Routine use of bone marrow examination in the diagnostic workup of isolated thrombocytopenia is not required in our center even if steroids are planned as a first line therapy. However, a detailed history, thorough examination with complete hemogram and peripheral smear examination are essential.

Pleural effusions in acute myeloid leukemia (AML) can have wide differential diagnosis, however AML presenting as pleural effusion with leukemic infiltration is rarely documented. A 22 year old male presented with pleural effusion for 3 months and subsequently diagnosed as AML M2, which prompted us for this communication.