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Systemic mastocytosis (SM) with associated clonal nonmast cell lineage disease is seen in up to 20% cases of SM. SM is uncommon in the pediatric population. T (8; 21) (q22; q22) is a good prognostic factor in acute myeloid leukemia (AML). However, the presence of SM confers poor prognosis in t (8; 21) (q22; q22) associated AML. We report the case of a child with t (8; 21) (q22; q22) associated AML with SM and her minimal residual disease status over the course of her treatment. In our case, the abnormal mast cells, showing co-expression of CD25 and CD2, persisted even after the marrow showed no evidence of residual AML.

Surface light chain expression is a feature of mature B-cell neoplasms. Light chain restriction in precursor B acute lymphoblastic leukemia is infrequently seen. We report a case of a 28-year-old female with non-FAB L3 morphology blasts and immunophenotypic features showing overlap between a precursor and mature B-cell neoplasm.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of acute leukemia that typically follows a highly aggressive clinical course in adults, whereas experience in children with this disease is very limited. We report cases of two children in whom bone marrow showed infiltration by large atypical monocytoid ′blast-like′ cells which on immunophenotyping expressed CD4, CD56, HLA-DR and CD33 while were negative for CD34 other T-cell, B-cell and myeloid markers. The differential diagnoses considered were AML, T/NK-cell leukemia and acute undifferentiated leukemia. Additional markers CD303/BDCA-2 and CD123 which are recently validated plasmacytoid dendritic cell markers were done which helped us clinch the diagnosis of this rare neoplasm. An accurate diagnosis of BPDCN is essential in order to provide prompt treatment. Due to its rarity and only recent recognition as a distinct clinicopathological entity, no standardized therapeutic approach has been established for BPDCN.

A variety of lymphoma types have been reported in patients being treated with anticonvulsant therapy. Non-Hodgkin lymphomas have been reported twice as frequently as Hodgkin lymphomas. Association of nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) with dilantin therapy is extremely uncommon. We report a case of Hodgkin lymphoma in a 25-year-old male patient who had been treated with diphenylhydantoin sodium for generalized tonic clonic seizures for 15 years. Patient presented with left cervical and axillary lymphadenopathy persisting for more than 2 years after cessation of treatment with diphenylhydantoin. Computerized tomography scan of thorax, abdomen and pelvis revealed no significant lymphadenopathy or any organomegaly. Diagnosis of NLPHL was made on excision biopsy of the cervical lymph node. Although the association between diphenylhydantoin therapy and the development of immunosuppression and lymphoma is well-documented, the role of the drug in the etiology of these disorders is still controversial.

Manifestations of parvovirus B19 vary even in the normal host from asymptomatic or subclinical infection to a spectrum of illness with symptoms during viremic and immune complex mediated stage of disease. We report the morphological findings of parvovirus B19 infection (confirmed on serology) in a patient of T-acute lymphoblastic lymphoma (T-ALL) who underwent induction phase of chemotherapy (MCP 842 protocol). Persistent pancytopenia in the bone marrow aspirate with mild increase in blasts was thought to be due to failure to achieve marrow remission. However, giant pronormoblasts with prominent intranuclear inclusions confirmed on trephine biopsy led to the suspicion of parvovirus B19 infection which was later confirmed on serology. This case is presented to report the rarely seen classical morphological feature of parvovirus infection on bone marrow examination which was incidentally the first investigation to diagnose the viremic phase of the infection, indicating that a high index of suspicion needs to be kept in mind while examining bone marrows of susceptible patients.

Merkel cell carcinoma is a primary neuroendocrine carcinoma of the skin. It is a highly aggressive tumor which commonly metastasizes to lymph nodes, liver, lung and bone. The diagnosis is based on histology and immunohistochemistry. Renal metastasis, with sparing of other common sites of hematogenous spread (lung and liver), is a unique feature of this case.

Background: Angioimmunoblastic T-cell lymphoma (AITL), a subtype of peripheral T-cell lymphoma (PTCL), is characterized by unique clinical and biological features. Its diagnosis remains a challenge as clinical presentation as well as pathologic findings are frequently misleading. Material and Methods: We retrospectively analyzed the clinical, morphological and immunophenotypic spectrum of 17 cases of histologically proven AITL. Result: The mean age was 54 years and male to female ratio was 2.4. Common clinical features included generalized lymphadenopathy (60%), hepatomegaly (70%), splenomegaly (50%), anemia (80%) and polyclonal hypergammaglobulinemia (100%). Microscopically, three architectural patterns; pattern I (6%), pattern II (41%) and pattern III (53%) were observed. Bone marrow infiltration was seen in 60% cases and 30% cases revealed plasmacytosis. Absence of follicles, polymorphous infiltrate, extra-follicular follicular dendritic cell (FDC) proliferation, high endothelial venules (HEV) prominence and neoplastic T-cells were the diagnostic features of AITL. CD10 positivity (47%), clear cells in the background (59%) admixture with large size CD20+ B-immunoblasts (35%) and bone marrow plasmacytosis (50%) were common observations. Conclusion: Awareness of various morphological and immunophenotypic complexities of AITL and distinction from reactive adenopathies and other types of lymphomas that mimic AITL is underscored in this study.

Peripheral blood smear examination is the most specific as well as the most common test performed for the diagnosis of malaria. Schizonts, ring forms (trophozoites) and gametocytes are the stages of malarial parasite that are commonly seen in the peripheral blood smear of a patient. Here, we report an extremely rare case of a 40-year-old male patient who presented with Plasmodium vivax infection with multiple exflagellated microgametes in the peripheral blood smear with review of the literature. Exflagellation of microgametes in malarial parasites is only seen in the definitive host, mosquito, and is very unusual to see during the developmental phases in the intermediate host, human. It is important to recognize these exflagellated microgametes in the peripheral blood smear as they may lead to diagnostic confusion with organisms such as spirochetes and trypanosomes.

Dermatopathic lymphadenitis is a rare entity described in patients with Human immunodeficiency virus infection. Here we present a case of dermatopathic lymphadenitis in a 50-year-old female who was HIV positive and did not have any obvious skin lesions. Fine needle aspiration cytology of the lymphnode showed a lymphoproliferative lesion and a subsequent biopsy showed atypical lymphoid proliferation showing prominent T-zone. Immunohistochemistry showed features of dermatopathic lymphadenitis.

Flow cytometric detection of intracellular antigens has become a standard method in establishing proper leukemic cell lineage affiliation. It has a non-debatable contribution to the diagnosis of hematolymphoid neoplasm as well as in minimal residual disease. Combination of analysis of fluorescence labeling and light scatter properties of cells allows rapid and better determination of target cell antigens. Regarding the detection of intracellular antigens, standardization of the procedure remains, however, a real challenge. Detection of intracellular antigens by flow cytometry (FCM) requires effective fixation and permeabilization of the cell membrane. In the available literature, some reports describe methodologies to achieve satisfactory results for detection of either cytoplasmic or nuclear antigens; however, no methodological consensus has yet been achieved among the laboratories. This article is an attempt to describe different approaches to detect intracellular molecules by FCM.

Background: Immunophenotyping of hematolymphoid neoplasms is being done in many laboratories in India. The first national meeting on Guidelines for Immunophenotyping of Hematolymphoid Neoplasms by Flow Cytometry was held on 14 March 2008 in Mumbai, India. Aim: To achieve uniformity in the laboratory practice regarding antibody panel selection in diagnosing hematolymphoid neoplasms. Settings and Design: Members of the Inter-Laboratory Comparison Program (ILCP) group in Mumbai prepared a draft regarding immunophenotypic panel selection for acute leukemias (ALs) and chronic lymphoproliferative disorders (CLPDs), which was further circulated among national and international cytometrists, hematopathologists, and oncologists for their written inputs, suggestions, proposed modifications; as well as their indications, if any, of the recommendations not being acceptable. Practice-based questionnaire was circulated among all the participants. Results: Consensus was attained, and the panel recommended the use of a minimal screening panel, followed by a secondary directed panel. The aim of the minimal screening panel would be to provide a diagnosis of all commonly occurring hematolymphoid neoplasms without the need of additional antibodies in most cases. Conclusion: Thus we could attain a consensus for our guidelines in selecting panels for ALs and CLPDs. The guideline is an attempt to formulate a minimal panel for immunophenotyping of hematolymphoid neoplasms. Laboratories are encouraged to add additional antibodies to the above panel to increase the sensitivity; however, they should refrain from immunophenotyping with fewer antibodies. This national guideline hopefully brings about uniformity and comparability in reporting of leukemia and lymphoma and bridges the divide between low-cost reporting and an accurate diagnosis.

Background: Isolated primary pancreatic lymphoma (PPL) is a rare extra-lymphatic non-Hodgkin's lymphoma comprising less than 1% of all extra-lymphatic lymphomas. It is seen in people of advanced age and there is a slight male preponderance. It is difficult to diagnose; the vague presenting symptoms and nonspecific laboratory/radiological findings make it difficult to differentiate the condition from pancreatic adenocarcinoma. Histopathological examination is of paramount importance to conclusively establish the diagnosis since the treatment involves lymphoma protocols, and prognosis and survival in PPL are considerably superior to that in adenocarcinoma pancreas. Case Report: We report a case of isolated PPL diagnosed after Tru-Cut biopsy and immunohistochemistry after a thorough staging workup. Result: The patient was treated with multi-agent combination chemotherapy followed by radiotherapy. Discussion: A review of literature was done using a Medline search to determine the incidence and prevalence of isolated PPL and to note the diagnosis and management of previously reported cases. Conclusion: An exceedingly rare entity, isolated PPLs need to be differentiated from pancreatic adenocarcinomas by histopathological evaluation since management is on the lines of other extralymphatic lymphomas and prognosis is significantly better.