A 35-year-old woman with background of liver cirrhosis and portal hypertension secondary to chronic hepatitis C presented with complication of hypersplenism and thrombocytopenia. She developed severe menorrhagia requiring multiple blood transfusions. In addition, her interferon therapy was withheld owing to the underlying thrombocytopenia. Partial splenic embolisation was performed, which improved her platelet counts. Subsequently, the menorrhagia was resolved and her interferon therapy was restarted.
A young patient presenting with splenomegaly and hypersplenism was inadvertently found to have selective IgA deficiency. There were no symptoms of immunodeficiency and the patient responded well to splenectomy, with return of blood counts to normal without adverse effects. No other cause for the hypersplenism was found. We postulate selective IgA deficiency as a cause of splenomegaly and hypersplenism.
Two patients with gout associated with the presence of an abnormal hemoglobin, Hb E, and hypersplenism are presented. Very large sclerotic-rimmed cystic erosions in the sacroiliac joints of both patients are unusual but characteristic of the skeletal lesions of gout. The hyperuricemia may be the result of the disordered nucleic acid metabolism associated with hemoglobin abnormality. The development of hypersplenism very likely accelerated this process and resulted in the clinical and radiographic manifestations of severe gout.
INDEX TERMS: Blood, diseases • Blood, proteins • globin and Hemoglobin Compounds • Sacroiliac Joint trophy
Study site: Hospital Gombak, Selangor, Malaysia
A 47-year old man had hypersplenism from massive splenomegaly, the cause of which was undetermined for 2 years. He was initially asymptomatic though there was mild pancytopenia. However, 18 months after presentation he manifested both clinical and haematological deterioration, almost succumbing to sepsis. Splenectomy finally provided a definite diagnosis of follicular lymphoma and also restored his blood counts to within normal range.
Familial myelodysplastic syndrome occurring at a young age is a very rare childhood haematological malignancy. Two siblings, aged three and 18 years, from a consanguineous marriage, presented with pancytopenia and was subsequently diagnosed to have myelodysplastic syndrome. Both remained clinically stable throughout the illness. Splenectomy appeared to have fully corrected the cytopenia in one of them.
Non-cirrhotic portal hypertension (NCPH) is clinically defined as the presence of portal hypertension in the background of non cirrhotic liver. It is diagnosed by the findings in ultrasound of the hepatobiliary system and also oesophagogastroduodenoscopy (OGDS) that consistent with that of a portal hypertension, but otherwise has a relatively normal liver function and echotexture. The treatment mainly focuses on primary and secondary prophylaxis of variceal bleeding both pharmacologically like non-selective beta-blockers and octreotide, and non-pharmacologically like endoscopic band ligation of varices and sclerotherapy. In advance cases, sometimes surgery such as Porto systemic shunt or splenectomy may be required especially in patients with uncontrolled variceal bleeding or with symptomatic hypersplenism. Here we report a case of a young man who presented with upper gastro-intestinal bleeding, which was initially thought from a bleeding ulcer but was found to be secondary to oesophageal and gastro-oesophageal varices. Apart from having mild ascites, he has no other features of portal hypertension. His liver biochemistry and echotexture were also normal. Unfortunately, the patient was lost to follow up while he was still in the early stage of investigating the condition. The purpose of this case report is to share an uncommon occurrence of NCPH in East Malaysia, where liver cirrhosis predominates the aetiology of portal hypertension. Also, to the best of our knowledge, there is a very limited reporting of a similar case in this region.
Myelofibrosis is a chronic myeloproliferative disorder characterised by splenomegaly, leukoerythroblastic anaemia, tear-drop poikilocytosis and marrow fibrosis. Splenectomy is indicated for patients requiring frequent transfusions or massive splenomegaly causing distressing symptoms and severe thrombocytopenia secondary to hypersplenism.A 56 year-old lady diagnosed to have primary myelofibrosis in year 2000 was referred to HUKM for further management of her increasing requirement of blood transfusion and massive splenomegaly. She has had two episodes of acute cholecytitis. Investigations done in our hospital showed that her hemoglobin level was 6.4g/dl, white cell count of 23.4x109/l, platelet count 163x109/l and peripheral blood showing leukoerythroblastic picture and tear-drop poikilocytosis. Leukocyte alkaline phosphatase score (NAP) was 184/100 neutrophils. Ultrasound of the abdomen showed massive splenomegaly with multiple gall stones. In view of the frequent transfusion requirements and symptomatic massive splenomegaly, splenectomy and cholecystectomy was performed. A review of her peripheral blood picture, post-splenectomy, showed marked reduction of the tear-drop poikilocytosis and leukoerythroblastosis. We believe that the spleen plays an important role in their formation, but the exact mechanism remains unclear.