Gurjeevan Garewal

High prevalence of hyperhomocysteinemia in young population of North India - A potential risk factor for coronary artery disease?

Total number of study subjects 50 67 Age range 26–40 26–40 Male:Female ratio 4.5:1 4.5:1 Subjects with normal fHcy 15 (30.0%) 22 (32.8%) Subjects with HHC 35 (70.0%) 45 (67.2%) Number of individuals given methionine load 50 27 HHC after methionine load (PML unmasking) 39/50 (78.0%) 22/27 (81.5%) MTHFR heterozygote 17 (34.0%) 9 (33.3%) Individuals given vitamin supplements 49 20 Hyperhomocysteinemic individuals benefited by vitamin supplements 20/35 (57.1%) 11/20 (55.0%)

Sweet's syndrome in association with myelodysplastic syndrome

No toxicity was encountered during the three courses of GM-CSF treatment, except a low grade fever with a maximum of 38 O C which responded to paracetamol. The patient died 1 month after the last course, from intracerebral bleeding and systemic fungal infection caused by bone marrow aplasia. Bone marrow recovery after a toxic insult appears to depend on available progenitor cells and growth factors. Interestingly, deficiency of growth factors seems to limit bone marrow recovery after regulardose chemotherapy since infusion of exogenous CSFs shortens the duration of aplasia after various chemotherapy regimens and autologous bone marrow transplantation ( 2 , 5 ) . Until now, no exhaustion of bone marrow reserve has been described even after repeated cycles of chemotherapy followed by

Clinico-Hematological Profile and Natural History of Childhood Myelodysplastic Syndromes

The clinical and hematological characteristics of ten children with myelodysplastic syndromes diagnosed and followed up over a 3 year period are presented. All of them had anemia and a low platelet count whilst the white blood cell count was variable. Presentation with bilateral proptosis and acute febrile neutrophilic dermatosis (Sweet’s syndrome) were unique features observed in one case each. None of these cases could afford specific therapy and thus serve to illustrate the natural history of the disease in pediatric practice.

Colchicine therapy in immune thrombocytopenic purpura.

In children with immune thrombocytopenic purpura (ITP), complete recovery occurs in up to 90% within six months, with or without conventional steroid treatment ( 1 , 2). However, 7-10% of paediatric patients pursue a chronic course (3). The management of these children with chronic refractory ITP is controversial. Suggested therapeutic options have varied from non-treatment to splenectomy or use of immunosuppressives, cytotoxic drugs, danazol and high doses of immunoglobulins (3-7). None of these agents have shown consistent results to date and serious side-effects have been noted with some. Recently, Strother et al. have reported good results when using colchicine in adults with ITP (8). The relative freedom from side-effects prompted us to use colchicine in children with chronic ITP and the experience is shared here.

Flowcytometric detection of PNH defect and response to therapy in aplastic anemia patients

Clonal hemopoietic stem cell disorders, characterized by features of bone marrow failure, include aplastic anemia (AA) and paroxysmal nocturnal hemoglobinuria (PNH) [1]. These diseases differ in the etiopathogenesis and clinical presentations, although treatment options for both still remain a clinician’s nightmare. Damashek, way back in 1960s, first appreciated the common link between AA and PNH i.e., one disorder can evolve into the other. The ‘PNH defect’, whether de novo or secondary (arising in the background of other hematological disorders like AA) is characterized by deficiency or complete lack of more than 20 different membrane proteins, which are covalently attached to the cell membrane via a glycosyl phosphatidyl inositol (GPI) anchor [2]. The absence of GPI anchored complement regulating proteins such as membrane inhibitor of reactive lysis (MIRL or CD 59) and decay accelerating factor (DAF or CD55) is responsible for the phenotype of the disease and same feature is used for diagnostic purposes as well [2]. There is scarcity of data in the literature regarding the frequency and prognostic implications of PNH defect in AA patients [2–4]. Here we elaborate upon our experience on this subject, published earlier as a preliminary communication [4].