Prospective evaluation of variables affecting platelet function in patients with newly diagnosed chronic myeloid leukemia.

: Platelet function in chronic myeloid leukemia (CML) could be affected by either hyperleucocytosis, clonal megakaryopoiesis, or tyrosine kinase inhibitors. However, these variables have never been prospectively evaluated. We conducted a prospective study over a period of 1.5 years in a tertiary care center of north India. Patients with CML in chronic phase, more than 18 years, and treated with imatinib were enrolled (n = 32). Age, and sex-matched controls were also included. Platelet function test was performed using two-channel Chrono-Log aggregometer 490 at four time-points: first, at diagnosis; second, after leucoreduction (total leucocyte count, <10 × 10/l) achieved with hydroxycarbamide; third, on-imatinib at BCR-ABL less than 1%; and fourth, in an independent cohort (off-imatinib) at deep molecular response (DMR) (BCR-ABL < 0.01%). Statistical analysis was performed using IBM SPSS statistics (version 22.0). Median age of patients was 42 years (15-65), and M : F ratio was 1 : 1. At diagnosis, platelet function correlated negatively with total leucocyte count, but not with platelet count. As compared with baseline, platelet aggregation with ADP (2.5 µl), and collagen (2.5 µl) improved significantly after leucoreduction (P = 0.05 and 0.009, respectively). Imatinib further caused significant impairment of aggregation with ADP (2.5 µl), collagen (2.5 µl), and collagen (1 µl) (P = 0.04, 0.008, and 0.02, respectively). Patients in DMR also demonstrated a significant impairment of platelet aggregation with all the agonists as compared with controls. While leucoreduction alone can improve the baseline platelet function derangement in CML, imatinib further impairs it. Residual CML stem cells, or effect of imatinib on normal common myeloid progenitors might account for platelet function derangement at DMR.

Study of Prothrombotic Changes in Metabolic Syndrome.

The metabolic syndrome is a complex disorder of various metabolic risk factors in a single individual having central obesity and commonly associated with diabetes and cardiovascular diseases. The aim of our study was to study the relationship between coagulation abnormalities and metabolic syndrome. We performed a prospective cross-sectional study in a tertiary care hospital. A total of fifty cases of metabolic syndrome and fifty age & sex matched controls were selected. These two groups were investigated for Prothrombin Time (PT), Activated Partial Thromboplastin Time (APTT), Fibrinogen levels, Plasminogen Activator Inhibitor 1(PAI1) levels and Factor VIII levels. In cases with metabolic syndrome, significantly increased levels of Fibrinogen, Factor VIII and Plasminogen Activator Inhibitor1 (PAI1) were observed. PT & APTT were shorter in cases with metabolic syndrome. The coagulation parameters studied, correlated significantly with the components of metabolic syndrome. Metabolic syndrome is a hypercoagulable state and further studies are required for further evaluation of the consequences of this hypercoagulable state. There is a need for clinical trials evaluating prophylactic anticoagulation for prevention of venous thrombosis in patients with metabolic syndrome.

Utilisation of Flow-cytometry in the Diagnosis of Auto Immune Haemolytic Anaemia.

Auto Immune Haemolytic Anaemia (AIHA) is one of the most common types of acquired haemolytic anaemias. Its main cause is auto-antibody mediated rapid destruction of Red Blood Cells (RBCs). Demonstration of a positive Direct Antiglobulin Test also known as Coomb's test, against these autoantibodies is the crucial serological assay in the diagnosis of AIHA. This routinely used test has the disadvantage of low sensitivity and does not detect low levels of red cell auto antibodies leading to false negative results sometimes. Flow cytometry can effectively diagnose such patients with low levels of autoantibodies. This study was carried out in a tertiary care center, where patients with suspected AIHA were studied during 2 years period. Blood samples of suspected patients of AIHA were tested by both Gel Card Test and by Flow-cytometry for detection of RBC bound IgG. A total of 50 patients with suspected diagnosis of AIHA were studied by flow-cytometry as well as by Gel card test for detection of RBC bound IgG. Out of these 50 cases, 41 cases have turned out to be positive and 9 were negative by flow-cytometry. By Gel card test, out of 50 cases, 34 were positive and 16 were negative. Therefore, there were 7 cases which were negative for RBC bound IgG by Gel card test and these were positive by flow-cytometry. Flow-cytometry is a reliable and more sensitive method and can be used as a new routine diagnostic technique for AIHA.

The paradox of recurrent with rare: A rare case of bilateral proptosis and facial palsy in acute myeloid leukemia with recurrent cytogenetic translocation t(8:21).

A 13-year-old female child presented with bilateral proptosis and subconjuctival hemorrhage of 2 months duration. Subsequently, the biopsy and peripheral smears confirmed the diagnosis of acute myeloid leukemia (AML). Recurrent cytogenetic translocation t(8:21) in AML associated with extramedullary manifestation; was discovered which is a rare event. Furthermore, myeloperoxidase negative blasts and periodic acid Schiff negative blasts in AML with t(8:21) is a very rare combination to the best of our knowledge.

Haematological characterisation and molecular basis of asian Indian inversion deletions delta Beta thalassemia: a case report.

The hereditary persistence of fetal hemoglobin (HPFH) and delta beta thalassemia are heterogeneous disorders characterised by increased levels of fetal hemoglobin and high level of this Hb continues in adulthood. The distinction between these two conditions is not always possible with routine hematologic analysis and molecular characterisation of the defect is required. We encountered such a rare case of δ ß thalassemia in a 10-year-old male child who presented with features of thalassemia intermedia. Hemoglobin analysis showed 100% HbF while molecular analysis revealed Asian Indian inversion-deletion GγAγ(δ ß) zero thalassemia.

Mycosis fungoides: A case report.

Mycosis fungoides is the commonest cutaneous T cell lymphoma. Clinically it is characterized by patch, plaque, tumor nodules; erythrodermic and poikoilodermous stages which may overlap. Extracutaneous spread takes place in late stages where any organ may be involved. The majority of reported cases are in adult males. A rare case of mycosis fungoides in a 38-year-old female is described. She presented in advance stage of disease with nodules all over the body.

Multiple myeloma presenting as primary non-secretory plasma cell leukemia.

Primary plasma cell leukemia is a rare manifestation of multiple myeloma, whose neoplastic hierarchy in the classification of malignant hematological disorders is not yet very clearly defined. Morphological and immunological criteria indicate that the cells are at end stage of B cell maturation pathway. This unusual disorder is diagnosed by the presence of more than 2 x 10(9) plasma cells per liter of peripheral blood or more than 20% of the leucocytes being plasma cells on differential count. This occurs either denovo or as a terminal event in patients with long standing multiple myeloma. A case report of a young male patient with primary plasma cell leukemia is presented.

Pancytopenia--a clinico haematological study of 200 cases.

The present study was designed to ascertain the percentage of occurrence and causes of pancytopenia. All the cases of pancytopenia from July 2001 to June 2002 (one year) were examined in the Department of haematology, Safdarjung Hospital, New Delhi. Bone marrow aspirations/biopsy were performed in most of the cases (200 out of 250 cases). The commonest cause of pancytopenia, in our hospital was Megaloblastic anaemia (72%), followed by Aplastic anaemia (14%) and others.