Paroxysmal nocturnal haemoglobinuria with Budd Chiari syndrome and Myelofibrosis 764-767
Dr. Mushtak Talib Abbas (MD) (MRCP), Senior Specialist/ Department of Medicine Hamad general Hospital/Doha-Qatar.E Mail: email@example.com, PO Box 3050.Tel: 009745220486, Fax 009744392273
We report a case of paroxysmal nocturnal haemoglobinuria (PNH) in a 38-year-old Indian female, who was admitted to our hospital with abdominal distension of 2-weeks duration, with leg oedema and jaundice. Her medical history was remarkable for deep vein thrombosis 15 years back, and stroke, nine years before admission. On clinical examination, spleen and liver were palpable, along with evidence of shifting dullness on abdominal percussion. Bone marrow examination showed hypoplastic bone marrow with agnogenic myeloid metaplasia, with myelofibrosis. Acidified serum lysis (Ham test) and sucrose lysis tests were strongly positive. The diagnosis of PNH was confirmed by flow cytometry. The patient could not be followed further, as she left for her country.