Giant Splenomegaly Research Articles

Splenectomy for a case with Waldenström macroglobulinemia with giant splenomegaly

Splenectomy for a case with Waldenström macroglobulinemia with giant splenomegaly

AIDS with Fever, Giant Splenomegaly and Severe Pancytopenia

AIDS with Fever, Giant Splenomegaly and Severe Pancytopenia

成人型Ｇａｕｃｈｅｒ病の１剖検例

An autopsy case of Gaucher's disease (adult type) was reported. A 39-years-old man was admitted to our hospital because of dyspnea. Splenomegaly was documented when he was 3-years-old. Physical examination revealed generalized edema, kyphoscoliosis and giant splenomegaly. Any abnormal neurological findings could not detected. Bone marrow aspiration biopsy revealed several clusters of Gaucher cells with “wrinkled tissue paper” like cytoplasm and round eccentric nucleus. The acid phosphatase activity in the serum increased to 38.5u/l, whereas the angiotensin converting enzyme activity was normal, perhaps because of steroid therapy. Inspite of intensive treatement, he died of respiratory failure.Autopsy findings revealed that Gaucher cells had proliferated in spleen, liver, bone marrow and lungs. Histochemically, these cells were positive stainini for acid phosphatase, PAS, Naphthol-AS-D-chloroacetate and negative for lysozyme. Electron microscopically, characteristic spindle-shaped and membrane-delimitted inclusion bodies were observed within the cytoplasm of the cells.Biochemically, the amount of glucocerebroside extracted from autopsy liver and spleen was greatly increased and the activity of glucocerebrosidase in his cultured skin fibroblasts were markedly low comparing with normal volunteers, confirming that the defect of this enzyme was the cause of this disorder.In Japan there were only eight cases of this disease (adult type) reported.

PRECURSOR T CELL LEUKEMIA

A case of acute lymphoblastic leukemia with a giant splenomegaly in an 8‐year‐old boy was investigated by immunological, cytochemical and electronmicroscopical techniques. Bone marrow and peripheral blood were largely replaced by large blast cells with a nonconvoluted nucleus. Cytochemically, most of the blast cells showed strong focal acid phosphatase activity. In the surface marker studies, these blast cells formed EAC rosettes but not E rosettes, while they showed positive reactivity with anti‐T lymphocyte serum but not with anti‐B lymphocyte serum. This membrane phenotype E‐, C3R+, T+, B‐ suggested that leukemic blast cells in this patient presumably originated from precursor T cells.

A Case of Pernicious Anemia with Giant Splenomegaly

A Case of Pernicious Anemia with Giant Splenomegaly

Giant Splenomegaly in Sarcoidosis Demonstrated by Radionuclide Scintiphotography

Massive enlargement of the spleen in a patient with disseminated sarcoidosis and the response of the patient to therapy were demonstrated by serial radioactive nuclide scintiphotographs.

Giant splenomegaly in sarcoidosis demonstrated by radionuclide scintiphotography. Response to corticosteroid therapy

Giant splenomegaly in sarcoidosis demonstrated by radionuclide scintiphotography. Response to corticosteroid therapy

Arteriographic studies of splenic and hepatic arteries in portal hypertension.

The radii of the splenic and proper hepatic arteries were estimated by means of selective celiac arteriography in cases of portal hypertension and of splenomegaly without portal hypertension. The results were evaluated in comparison with normal arteriograms. The estimated radii of the splenic arteries were in satisfactory agreement with the histometrical ones. It was concluded that the splenic and hepatic arterial flow could be proportional to the 2.7th power of the radii of the respective arteries on arteriograms, as in the histometrical study.1, 2 In cases of portal hypertension and appreciable increase in the radius of the splenic artery was demonstrated. In splenomegaly without portal hypertension, as in idiopathic thrombocytopenic purpura, hemolytic jaundice, such an increase in the radius of the splenic artery was not observed. In portal hypertension, the radius of the splenic artery could be correlated with the splenic weight, portal pressure, the grade of decrease in the portal pressure after splenectomy and the length of the splenic artery. In cases of liver fibrosis, dilatation of the splenic artery was not usually accompanied by dilatation of the proper hepatic artery, whereas in liver cirrhosis with or without giant splenomegaly, dilatation was noted not only in the splenic artery but also in the proper hepatic artery as well.