An analysis of 30 cases of myelodysplastic syndrome.

Myelodysplastic syndrome (MDS) is a clonal disorder of pluripotential stem cells of the bone marrow. The purpose of the study was to obtain epidemiological data of MDS. Thirty cases of MDS presented from April 1998 to May 2006 are included in this study. Complete blood counts were performed in an automated cell counter. Bone marrow aspiration, trephine biopsy and chromosomal study were carried out. The dry film was stained using a Romanwasky stain and Perl's stain. Fifteen cases were male and 15 cases were female. The mean age at presentation was 55 years (range 8-73 years). A majority of the patients presented with weakness (63.33%). Autoimmune manifestations in the form of joint pain were present in 13.33%. Patients were symptomatic for a prolonged period before diagnosis could be reached (average 358.8 days). A majority of the patients had MDS-refractory anemia (MDS-RA) or MDS-RA with excess blasts (MDS-RAEB-2) at presentation. Three patients had chromosomal abnormalities (27.27%). Eight patients (26.7%) were relatively young at presentation, less than 50 years of age. Three (10%) were children. A majority of the patients opted for symptomatic treatment only.

An analysis of 8 cases of factor X deficiency.

BACKGROUND: Factor X deficiency is a rare coagulation defect. There are occasional reports of factor X deficiency from India. Difficulty in accurate diagnosis and non-availability of ideal treatment is discussed. METHODS: Eight cases of factor X deficiency, diagnosed from 1992 to 2007 are reported here RESULTS: Seven were male while one was female. Seven patients were symptomatic from early childhood. One patient became symptomatic from 18 years of age. Factor X assay was done in 4 patients, 3 had severe deficiency and one had mild deficiency. One patient had associated factor IX deficiency. Three patients had repeated bleeding episodes requiring multiple transfusions. Two patients had intracranial bleed and one had umbilical cord bleeding at birth. There was no mortality. No patient received prophylactic transfusion. CONCLUSIONS: Factor X deficiency is a rare coagulation defect. Hereditary deficiency should be distinguished from acquired deficiency. CNS, joints and skin are the common sites of bleeding.