Sajad Geelani

Hereditary stomatocytosis: First case report from Valley of Kashmir

Stomatocytes are erythrocytes with a central slit or mouth-shaped (stoma) area of central pallor when examined on dried smears. In wet preparations, they are uniconcave rather than biconcave, giving them a bowllike appearance. In vitro, stomatocytes are produced by drugs that intercalate into the inner half of the lipid bilayer, thereby expanding the inner lipid surface area relative to that of the outer half of the bilayer. Hereditary stomatocytosis (also known as hereditary hydrocytosis, or overhydrated stomatocytosis) refers to a heterogeneous group of autosomal dominant hemolytic anemias caused by altered sodium permeability of the red cell membrane. We present the first case report of hereditary stomatocytosis in a 10-year-old male from the valley of Kashmir. Only eight families with this condition have been described worldwide.

PLASMAPHERESIS: AN EXPERIENCE AT A TERTIARY CARE HOSPITAL.

Nusrat Bashir. Objectives:To analyze our experience with 105 cases who underwent plasmapheresis for various disorders. Materials and Methods:-Retrospective review of Plasmapheresis(PE) was done over a period of 13 years, from july 2015 to August 2002 in a tertiary care centre. Results:The main indication for PE was GuillainBarresyndrome( GBS) ( 31%) . Age of patients ranged from 18-62 years. The most common complications were paraesthesias and/or cramps (36.1%) and hypocalcemia (7%).There was no mortality related to the procedure. Conclusion:-The analysis of 105 cases of plasmapheresis in our department showed that the procedure is safe, with only minimal procedure related complications and no mortality.

Cured case of relapsed acute myeloid leukemia with giant central nervous system chloroma

Myeloid leukemias are a heterogenous group of disease characterized by infiltration of blood, bone marrow and other tissues by neoplastic cells of hematopoietic origin. Rarely, patients may present with symptoms from a mass lesion located in soft tissues. The mass lesion represents a tumor of leukemic cells and is called granulocytic sarcoma or chloroma. This is more commonly seen in monocytic subtype with abnormalities of chromosome 11. In general, they are felt to augur a poorer prognosis, with a poorer response to treatment and worse survival. There have been few case reports of orbital and central nervous system (CNS) chloroma in pediatric age group who have done better than only a medullary disease. Here, we present a case of acute myelogenous leukemia M2 who had two relapses one medullary and one extramedullary (CNS relapse with giant CNS chloroma). Patient was reinduced with same drugs and was advised to go for allogenic stem cell transplantation, which he could not. Subsequently presented with features of raised  Intracranial tension (ICT) and was diagnosed to have extramedullary relapse in the form of giant CNS chloroma. He was treated with surgical debulking of the tumor, fludarabine, AraC, idarubicin, and G-CSF chemotherapy protocol (FLAG-IDA) chemo, and local intraspinal triple therapy 5 years back and stands cured at present.