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Type 1 diabetes mellitus (T1DM) is a common autoimmune pathology requiring lifelong insulin therapy. We report the case of a 12-year-old girl with T1DM admitted to Department C of the National Institute of Nutrition of Tunis for diabetic ketosis. She had suffered from T1DM for five years, with poor glycemic control (hemoglobin A1C = 10%) and poor therapeutic adherence. On examination, she had abdominal bloating with homogeneous hepatomegaly. Her height was 146 cm (less than the second percentile), her weight was 38 kg (less than the second percentile), and her body mass index was 17.8 kg/m². Tanner's stage was S1P1A1. Biological investigations showed mixed dyslipidemia, normal liver and renal functions, and normal thyroid-stimulating hormone levels. The aspartate aminotransferase/alanine aminotransferase ratio was 1.35. Ultrasound of the abdomen revealed hepatomegaly with a liver span of 19 cm. Based on the clinical history and investigations, Mauriac syndrome was the most likely diagnosis of our patient. A holistic multidisciplinary approach, in collaboration with the child psychiatrist, was opted to optimize diabetes management and reduce hepatic metabolic overload. Further investigations were conducted to rule out differential diagnoses, especially viral and autoimmune hepatitis. Poor acceptance of type 1 diabetes leads to non-compliance with insulin therapy. Then, energy metabolism becomes defective with growth retardation and pubertal delay. Glucose accumulates in the liver leading to metabolic liver disease. Liver damage could be irreversible. Therapeutic education, a good doctor-patient relationship, and family support are the cornerstones of managing T1DM diabetes complicated by Mauriac syndrome., Competing Interests: Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Jemai et al.)

Introduction: Imatinib is a first-line selective tyrosine kinase inhibitor used for the treatment of chronic myeloid leukemia. Although imatinib-induced hepatotoxicity may aggravate the patient's clinical condition and alter the treatment plan, the mechanism of imatinib-induced hepatotoxicity has rarely been investigated., Case Report: We report a 51-year-old man, suffering from acute toxic hepatitis after 5 months of imatinib treatment for chronic myeloid leukemia., Management and Outcome: The outcome was favorable after discontinuation of treatment with normalization of biological liver function after 12 weeks. The treatment was switched to nilotinib without any incidents., Discussion: Regular liver function test monitoring is recommended during imatinib treatment. In fact of acute hepatic toxicity, treatment with imatinib should be stopped in the case of cytolysis more than five times the upper limit of normal., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Cerebral venous thrombosis is a rare consequence of lumbar punctures for intrathecal therapy. We report a patient treated for diffuse large B cell lymphoma with cerebral venous thrombosis after intrathecal Methotrexate administration. In this patient, intrathecal treatment was discontinued and he was successfully treated with high-dose low-molecular-weight heparin subcutaneously. Haematologist must be aware about neurological symptoms of cerebral venous thrombosis as a complication of lumbar puncture especially among patients with high coagulopathy state. If neurological symptom occurs, patient should be referred early to neurologist to avoid fatal outcome and neurological deficit.

Hemophilia is a rare constitutional hemorrhagic disorder. There is insufficient epidemiological data on hemophilia in Tunisia. To describe the epidemiological, clinical, therapeutic, and outcome of a cohort of patients with hemophilia in southern Tunisia. A retrospective study was conducted on patients with hemophilia at the Hemophilia Treatment Center of Southern Tunisia in Sfax over 38 years (from January 1982 to December 2020). Data were collected in a regional hemophilia registry of the South Tunisian center. We collected 141 cases of hemophilia, 85% of whom had hemophilia A and 15% had hemophilia B. The severe form represented 65%, followed by the moderate form at 25%. The prevalence of hemophilia was 4.4 in 100 000 population. Family history of hemophilia was found in 70%. The mean age of patients at diagnosis was 28 months. Hemophilia was detected in 87% of cases after hemorrhagic syndrome. Bleeding occurred mainly in hemarthrosis (73%), hematoma (70%), and visceral bleeding (28%). Intracranial bleeding occurred in 6% of cases. Thirty-six percent of patients were on prophylactic therapy. Hemophilic arthropathy was the most important orthopedic complication in our patients (38%). Inhibitory antibodies occurred in 16% of PWH. Transfusion-transmitted infections with HIV and hepatitis C were in 2 and 31% of cases, respectively. The prevalence of hemophilia is still underestimated in our center. The severe form of hemophilia is the most frequent. Hemophilic arthropathy was the most important complication in our patients. This showed that hemophilia is still a disabling disease in our country., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Primary plasma leukemia is defined by the presence of more than 20% plasma cells in the peripheral blood or number of circulating plasma cells greater than 2G/L. It has points in common with multiple myeloma and has certain characteristics, in particular its aggressiveness and poor prognosis. Through 02 cases diagnosed in the flow cytometry laboratory, the authors present the clinical, cytological and especially immunophenotypic features of this disease, with the emphasis on the role of flow cytometry in the diagnosis.

Introduction: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a proliferation of plasmacytoid cell precursors. It is a rare and difficult-to-diagnose hematological malignancy with a poor outcome., Case Presentation: We report three cases of BPDCN diagnosed in patients of different nationalities (Tunisian, Algerian and Libyan) and varying ages (eight, 65 and 74 years old). Cutaneous involvement was present in all three cases. Cytology was inconclusive in the first case, in favor of acute lymphoblastic leukemia (ALL) and acute myeloblastic leukemia (AML) in the second and third case respectively. The diagnosis was retained by flow cytometry, highlighting the Cluster of Differentiation (CD) 4 + CD56 + phenotype of the blast population., Conclusion: These observations illustrate diagnosis challenges, the importance of biological/clinical confrontation in order not to misdiagnose this entity. Flow cytometry is an essential diagnostic tool.

Introduction: B acute lymphoblastic leukaemia (B-ALL) is a proliferation of hematopoietic precursor cells characterized by the expression of various B-cell antigens. Expression of T cell antigens has rarely been reported in B-ALL. We report the second case CD5+ (Cluster of differentiation 5) B-ALL associated with Philadelphia chromosome (Phi+)., Observation: A 38-year old male presented with anorexia and generalized weakness for the last ten days. Hemogram revealed bicytopenia and hyperleukocytosis made of 93% difficult to classify cells. A diagnosis of diffuse large B-cell lymphoma was suspected. An immunophenotyping on peripheral blood was performed. The panel for B- cell lineage chronic lymphoproliferative disorders (B-CLPD) was used. The dim expression of CD45 and the lack of surface immunoglobuline helped to exclude a CD5 expressing mature B cell neoplasm. Then, the diagnosis of ALL was confirmed by ALL panels. Karyotype showed a Phi+. Thus, a diagnosis of B-ALL with aberrant expression of CD5 and Phi+ was established. The patient received chemotherapy according to the modified group for research on adult acute lymphoblastic leukemia philadelphia positive 2005 protocol (GRAAPH 2005). A complete remission at the end of induction was obtained. The patient received consolidation and then, hematopoietic stem cell transplantation. The patient is in complete hematological remission till the date of submission of this report., Conclusion: Aberrant expression of CD5 associated with Phi+ has rarely been reported in B cell lineage ALL and having a poor prognosis. Pathologists and clinicians should be aware of this entity to avoid confusion with other tumors.

Optic nerve infiltration is relatively rare in acute lymphoblastic leukemia. We present a case of a -53 year-old-man who was diagnosed with T- acute lymphoblastic leukemia (ALL). The patient was treated with ALL national protocol and the central nervous system (CNS) prophylactic management. On treatment, the patient presented with sudden severe vision deterioration of both eyes. Fundoscopic examination of the eye and magnetic resonance imaging of the orbits were in favor of an infiltration of the optical nerve. An isolated extramedullary relapse of the optical nerve was retained. The patient was treated with salvage chemotherapy systematic and intrathecal. Waiting forthe beginning of radiotherapy, the patient presented a bone marrow relapse. He died of a severe hemorrhagic syndrome. Conclusion: Optic nerve leukemic infiltration has a severe prognosis. Ophthalmic assessment is essential in patients with ALL in order to diagnose an early ocular involvement and the patient's vision can be preserved if treatment is initiated promptly.