Precursor lymphoblastic lymphoma in the extramedullary tissue: A rare manifestation of chronic myeloid leukemia in blast crisis

Chronic myeloid leukemia (CML) is a myeloproliferative disorder characterized by immature granulocytes in peripheral blood and bone marrow. In 95% of cases, it is always due to the presence of Philadelphia chromosome characterized by the presence of reciprocal translocation between chromosome 9 and 22. However, in 7% -17% of individuals, extramedullary proliferation also occurs, either in skin, lymph nodes, bone or central nervous system (CNS), which could be either myeloid, lymphoid or mixed progenitor in origin. The present case is of a 23-year-old male who presented with lower limb weakness, bowel and urinary incontinence. His complete blood count (CBC) findings showed a raised white blood count (WBC) of 408 X 10E9/L. Peripheral film, bone marrow biopsy and immunohistochemistry showed findings consistent with CML in chronic phase. Bone marrow cytogenetic revealed the presence of Philadelphia chromosome. Simultaneously, magnetic resonance imaging (MRI) was done which revealed extradural mass at L1-L3 level; histopathological and immunohistochemistry findings showed features compatible with precursor B cell lymphoblastic lymphoma. His cerebrospinal fluid (CSF) cytology revealed similar blast cells. This extramedullary presence of lymphoid blast cells in the CNS put the patient in the rare entity of CML in blast crisis. He was started on tablet nilotinib and also received multiple cycles of intrathecal chemotherapy with cytosar, methotrexate and hydrocortisone. He also underwent radiotherapy of extradural mass. His lower limb weakness improved dramatically. However, after receiving the fourth cycle of intrathecal therapy, the patient died consequent to neutropenic sepsis. Extramedullary blast crisis in CML has a poor prognosis. Any patient with CML, presenting with CNS symptoms or lymph node enlargement should be thoroughly investigated for extramedullary blast crisis, as there is a considerable change in management and prognosis from the prototype CML in chronic phase

A Pragmatic Approach to Superwarfarin Intoxication in a Resource Constraint Setting; a Case Report

Introduction: Superwarfarins are highly lethal rodenticides which are much more potent than the warfarin owing to various structural changes in the parent compound that confers high half-life and increase affinity to vitamin K epoxide. Case Presentation: We report a case of a 19 years old female who presented bleeding prolonged PT and APTT. Factor assay showed significantly lower levels vitamin K dependent coagulation factors. She was given conventional doses of FFP and vitamin K for two consecutive days, although she responded clinically but her coagulation profile failed to show any improvement. Hence superwarfarin poisoning was considered. Based on this assumption patient was cross questioned repeatedly, and subsequently she admitted that she had ingested rodenticide with the suicidal intent. Hence she was given higher doses of vitamin K and FFP for several days after which her PT and APTT shortened significantly. Discussion: Superwarfarin toxicity clinically presents with bleeding and deranged coagulation profile. Differentiating it from other causes is challenging but very crucial, as it does not responds to usual doses of vitamin K and plasma. Conclusion: This case highlights the importance of maintaining high index of suspicion of superwarfarin toxicity in coagulopathy of unknown etiology that fails to respond to conventional doses of treatment. Detailed history with interrogation of leading questions in such cases is of integral importance

Secondary haemophagocytosis in a patient with Ewing\u27s sarcoma

Secondary haemophagocytosis in malignant tumours have been reported in bone marrow. However we present engulfment of malignant apoptotic Ewing’s sarcoma cells by macrophages in the bone marrow. A 24 year old Pakistani male patient presented with complains of backache. After imaging, a laminectomy was performed the biopsy results of which were consistent with Ewing’s sarcoma. Subsequently for staging, bone marrow and trephine was done. The bone marrow aspirate showed markedly increased haemophagocytic activity with histiocytes engulfing malignant apoptotic Ewing’s sarcoma cells. The presence of histiocytes engulfing apoptotic Ewing’s sarcoma cells was of particular interest. Similar findings have rarely been reported

Clinico-hematological profile of chronic lymphoproliferative disorders in patients presenting with lymphocytosis

Objective: To assess the spectrum and clinico-haematological profile of chronic lymphoproliferative disorders in patients presenting with lymphocytosis. Method: The cross-sectional, retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised data related to cases of bone marrow aspirate and trephine from January to November 2020. Patients for whom the bone marrow was done for lymphocytosis were studied for the presence of lymphoproliferative disorders, sub-types and patients’ characteristics. The diagnosis and classification were based on the World Health Organisation criteria for tumours of haematopoietic and lymphoid tissues. Data was analysed using SPSS 21. Results: Of the 3,334 bone marrow specimens tested, 103(3%) were related to lymphocytosis. Of these, 84(82%) were diagnosed with lymphoproliferative disorders, while diagnosis remained undetermined in 19(18%) cases. Male:female ratio was 3.6:1 and median age was 60 years (range: 21-85 years). Constitutional symptoms were found in 61(73%) patients. Median absolute lymphocyte count was 45x109/L (range: 5.3-480). All 84(100%) patients were classified as B-cell lymphoproliferative disorder. Chronic lymphocytic leukaemia was the most common form, 61(73%), and 31(51%) of them presented with advanced stage disease. Conclusion: A huge majority of patients presenting with lymphocytosis had underlying lymphoproliferative disorders of which B-cell chronic lymphocytic leukaemia was found to be the most common. Key Words: Lymphoproliferative disorder, Lymphocytosis, Chronic lymphocytic leukaemia, Bone marrow, Pakistan

Use of ISTH bleeding assessment tool to predict inherited platelet dysfunction in resource constrained settings

Background: The International Society of Thrombosis & Hemostasis (ISTH) bleeding assessment tool (ISTH-BAT) is used to record bleeding symptoms in patients with possible bleeding disorders.AIM: To investigate the utility of the ISTH-BAT in predicting platelet dysfunction in individuals with suspected inherited platelet function disorders.METHOD: Individuals with clinical evidence of bleeding and suspected inherited platelet function disorder and healthy volunteers were included in the study. The ISTH-BAT questionnaire was applied prior to light transmission aggregometry (LTA).Results: A total of 261 participants were included (100 healthy volunteers, and 161 with suspected inherited platelet function disorders). The ISTH-BAT score in participants with suspected inherited platelet function disorders (median 2; interquartile range [IQR] 5-1) was significantly higher than in healthy volunteers (median 0; IQR 2-0). There was also a significant difference between participants with suspected inherited platelet function disorders with a platelet defect detected by LTA (median 4; IQR 8-3) and those with normal platelet function (median 2; IQR 3-1) (p\u3c0.001). The ISTH-BAT score was associated with a demonstrable platelet defect on platelet function testing (area under the receiver operating characteristic curve=0.8 [95% confidence interval 0.72-0.87, p=\u3c0.001] and odds ratio 3.25 [95% confidence interval 2.13-4.37, p=\u3c 0.001]).CONCLUSION: The ISTH-BAT is a useful tool for documenting bleeding symptoms and the score obtained is also predictive of the presence of a platelet defect on LTA in patients with suspected inherited platelet dysfunction

EVALUATION OF SPECTRUM OF LABORATORY HEMATOLOGICAL MANIFESTATIONS IN DENGUE FEVER

Dengue virus belongs to flavivirus family which gains entry into the host organism through skin following an infected mosquito bite. Humoral, cellular, and innate host immune responses are involved in the progression of the disease. Dengue fever is getting common in Pakistan, and at times shows high mortality, but there is limited literature available. Therefore, this study was designed to evaluate hematological parameters in patients with dengue fever. This was a retrospective cross-sectional study conducted in the Department of Microbiology, Dow Diagnostic Reference and Research Laboratory, Karachi, Pakistan from 1 January 2021 till December 2021. A total of 6140 were collected, out of which 1746 were found positive. Dengue infection was confirmed by rapid screening NS1 antigen by ICT method. IgM antibodies were detected by Enzyme linked immunosorbent assay (ELISA). Hematological analysis was performed on Sysmex analyzer. Among the 1746 positive samples, 1036 (60%) were males and 710 (40%) were females. More than 10 hemoglobin and 45 hematocrits were found in greater than 40% of cases. Leucopenia less than 4000 was observed mostly in age group 0-20 years. Eosinophilia, basophilia, lymphocytosis and atypical lymphocytosis were shown equally in all age groups. Our study found greater incidence of Dengue fever among 21-40 years of age group with male predominance. Hematological spectrum revealed thrombocytopenia, lymphocytosis, high hematocrit, eosinophilia, basophilia and monocytosis at the time of diagnosis