38 years old female with pluriglandular auto-immune syndrome (involvement of thyroid, adrenals, ovaries and history of miscarriage and muco-cutaneous candidiasis) initially presented to haematology service in 2002 with anaemia. Her blood count showed PLT 520 x10^9/L, WBC 4.5 x10^9/L, Neu 1.1 x10^9/L, Lym 3.1 x10^9/L, Hb 9.1 g/dl (supported). Initial bone marrow showed some dysplastic features. Repeated bone marrow in late 2002 showed increased granulopoiesis with normal maturation, lymphocytosis (11% of nucleated elements) but overall a normal bone marrow. She also had a mild splenomegaly. No cytogenetic analysis was performed. Patient was managed symptomatically with repeated RBC transfusions complicated by iron overload treated with desferoxamine. Manifestations of endocrine deficiencies were managed with supplementation of thyroxine, hydrocortisone and vitamin B12.
After about 2 years patient spontaneously become transfusion independent. In 2006 she again presented with neutropenia and anaemia. In 2007 FBC showed PLT 380 x10^9/L, WBC 16.1 x10^9/L, Neu 0.7 x10^9/L, Lym 14.8 x10^9/L, Hb 8.4 g/dl (supported), blood film showed presence of population of small lymphocytes. CT of thorax, abdomen and pelvis showed moderate splenomegaly. Autoantibody screen revealed: Intrinsic factor Ab – positive, TPO Abs - 76 (abnormal).
