Macrophage Activation Syndrome in a Patient with Systemic Lupus Erythematous Triggered by Tuberculous Meningitis.

Background: Hemophagocytic lymphohistiocytosis (HLH), is characterized by systemic uncontrolled inflammation resulting from immune dysregulation secondary to various triggers, including genetics, infections, autoimmune diseases, and malignancies. Macrophage activation syndrome (MAS) is an immune dysregulation phenomenon, in which an underlying rheumatological disease is present. We report a rare, interesting case of a middle-aged female, with a systemic lupus erythematosus (SLE) flare complicated by macrophage activation syndrome (MAS), in which tuberculous meningitis (TBM) was the identified trigger.

Acute monoblastic myeloid leukemic pleural effusion: Pitfalls and clues.

Acute myeloid leukemic pleural effusions are uncommon with heterogenous cytomorphology and variable immunoprofiles. This imposes a difficult cytologic diagnosis. In particular, acute myeloid leukemia of monocyte lineage mimicking benign and malignant lymphoid and non-lymphoid lesions is challenging.

Haematological & molecular profile of acute myelogenous leukaemia in India.

Background & Objective: Recurrent balanced translocations are generally recognized to be a major parameter for prognostication in acute myeloid leukaemia (AML). The chromosomal translocation t(15;17) results in PML/RARalpha fusion gene, t(8;21) results in AML1/ETO fusion gene and Inv 16 generates CBFbeta/MYH11 fusion gene. Patients with these mutations have a good prognosis unlike abnormalities in chromosome 5 or 7 or FLT3 genes.

Paroxysmal nocturnal hemoglobinuria in childhood and adolescence--a retrospective analysis of 18 cases.

Objective: To assess the clinical and hematological profile of PNH in children.

Methods: Clinical and laboratory features of children with PNH diagnosed in the past six years at our centre were reviewed. Various investigations done included a complete blood count and peripheral smear examination, plasma hemoglobin, urine hemosiderin, acid ham test, sucrose lysis test, immunophenotyping of erythrocytes by sephadex column gel card and of granulocytes by flow cytometry.

Does acute promyelocytic leukemia in Indian patients have biology different from the West?

Acute promyelocytic leukemia (APML) is a well-characterized malignancy with typical clinico-hematological and molecular features. However, Indian data on this malignancy are limited. This study was conducted to determine the clinico-hematological profile of APML in India.

AML1-ETO positive AML: first report from India.

Translocation (8;21) is associated with few typical morphological features and favorable prognosis. All patients of AML and MDS with increased blasts (N = 35) according to FAB criteria, presenting (between Jan 2004 to June 2005) to the Department of Hematology, AIIMS were studied. RT-PCR was done for the AML1-ETO fusion transcript in all cases.

HFE mutation H63D predicts risk of iron over load in thalassemia intermedia irrespective of blood transfusions.

Iron overload is a well-documented complication in thalassemia intermedia. Moreover, it is seen that the number of blood transfusions received does not correlate with the degree of overload. Since, HFE gene is associated with iron overload; the present study was conducted in an attempt to evaluate its role in thalassemia intermedia.

Impact of FLT3 internal tandem duplications on Indian acute promyelocytic leukemia patients: prognostic implications.

Despite recent advances in the treatment of acute promyelocytic leukemia (APL), early mortality and relapses still occur. With the view to evaluate the role of FLT3 mutation in APL, 54 patients (median age 28 years, range11-57 years, male to female ratio 1.2:1, median TLC 8.

Gel card in the diagnosis of autoimmune haemolytic anemia.

The diagnosis of autoimmune haemolytic anaemia (AIHA) requires the establishment of haemolysis and demonstration of autoantibodies against red cells. Most laboratories use the conventional Coomb's test for the demonstration of the autoantibodies. However, in approximately 2-6% of the patients who present with the clinical and haematological features of AIHA, the direct agglutination test is negative on repeated testing.

Congenital vitamin K-dependent coagulation factor deficiency: a case report.

Congenital vitamin K-dependent coagulation factor deficiency is a very rare bleeding disorder, which usually presents with episodes of intracerebral bleed in the first few weeks of life, sometimes leading to a fatal outcome. We report a case of combined factor deficiency of vitamin K-dependent factors in which the patient presented with both intracerebral bleeding, and possibly also thrombosis, and responded to a vitamin K supplement along with fresh frozen plasma.

Over-representation of bcr3 subtype of PML/RARalpha fusion gene in APL in Indian patients.

Thirty six patients with acute promyelocytic leukemia were studied by reverse transcriptase-polymerase chain reaction (RT-PCR) and real-time PCR. There was concordance between the results achieved by both the methods except in one case, which was negative by RT-PCR but positive by real-time PCR. The prevalence of bcr3 (short isoform) was found to be significantly higher than that of bcr1 (long isoform) (64 vs.

Disseminated intravascular coagulation as an unusual presentation of Kala-azar: report of two cases.

Kala-azar (visceral leishmaniasis) is a common problem in various well-defined areas of India. It is characterized by fever of long duration, enlarged liver and spleen, anaemia and leucopoenia. Bleeding is an uncommon manifestation of kala-azar.