Hyper eosinophilic syndrome; a case series

Hyper eosinophilic syndrome [HES] is a subset of idiopathic eosinophilia that fulfils the criteria of a persistent [>6 months] increase in absolute eosinophil count [AEC] [>1.5x10[9]/l] associated with target organ damage. Recently it was classified into myeloproliferative and lymphoid variants. Present study is aimed to study the clinico morphological features and variants of HES and their response to various therapeutic modalities. In Pakistani population. It is a case series conducted at Haematology department, Shaikh Zayed hospital, Lahore during 4 years from Jan 2005 to Dec 2008. This study included 8 adult patients of HES diagnosed on history, clinical features and elevated absolute eosinophil count [AEC]. Seven of 8 patients were of lymphoid-HES and 1 was of myeloproliferative-HES. M: F ratio is 7:1, mean age of presentation was 37 years in lymphoid- HES and 69 years in myeloproliferative -HES. Presenting features were fatigue, weight loss, fever, SOB, paraesthesia and skin rash. Mean AEC in l-HES was 16109/l and in myeloproliferative- HES was 22.7x109/l. Organ damage was seen in cardiovascular, gastrointestinal [GIT], respiratory and nervous systems. All of the lymphoid-HES responded to steroids. In conclusion, early diagnosis and targeted therapy improve outcome in HES

Clinico hematologic features of immune thrombocytopenic purpura and its association with autoimmune disorders

Immune thrombocytopenic purpura [ITP] is a clinical syndrome in which a decreased number of circulating platelets [thrombocytopaenia] manifests as a bleeding tendency, easy bruising [purpura], or extravasation of blood from capillaries into skin and mucous membranes [petechiae]. Present study was done to observe the clinico-haematological features of ITP in Adults and to analyse the association of autoimmune disorders with ITP in Pakistani patients. It was a cross-sectional descriptive study conducted at Shaikh Zayed Hospital, Lahore, from 1st January 2006 to 30[th] June 2007. The study included 44 adult patients of both genders diagnosed as having ITP according to WHO guidelines. Bone marrow biopsy was carried out in all patients and other causes of thrombocytopaenia were carefully excluded. Antinuclear antibodies, rheumatoid factor, HBs Ag, anti HCV, HIV were also done. The data was analysed by SPSS version 10. Results showed peak incidence in third decade with female to male ratio of 3.1:. Bleeding and bruising were common symptoms of ITP. Seven [15.9%] of 44 patients had serological evidence of systemic autoimmune disorders, i.e., SLE or RA. Platelet count was significantly lower in SLE patients than in entire cohort. It was concluded that adult ITP is predominantly seen in young females, presents with bleeding from more than 2 sites and may be associated with autoimmune disorders at the time of diagnosis

Hairy cell leukemia: a case report

We report a case of a middle aged male diagnosed as Hairy cell leukemia. Clinicohematologic features were fever, weight loss, splenomegaly, anemia and thrombocytopenia. Bone marrow and spleen were infilterated by Hairy cells. An unusual feature was high TLC with large number of Hairy cells resembling superficially Hairy cell Variant. But the Flow cytometric analysis confirmed that immunophenotype was that of classic Hairy cell leukemia. Patient responded to Cladarabine therapy and had an uneventful recovery