A case of histiocytic medullary reticulosis with splenic infaraction from a 23-year-old male is presented. Radiologic findings on selective spleen scintigraphy and abdominal CT are described. Selective spleen scintigraphy showed huge, multilobulated spleen with numerous photon-deficient areas in it and peripherally. Abdominal CT showed large peripheral band-like low density and infiltrative lesion in spleen with accompanying intraabdominal lymphadenopathy. Histoligical features were consistent with HMR in spleen and liver specimens.
Adult ; Hemoperitoneum/etiology ; Hepatomegaly/etiology/pathology ; Histiocytic Sarcoma/*complications ; Humans ; Male ; Splenic Infarction/*etiology/radiography/radionuclide imaging ; Splenic Rupture/etiology ; Splenomegaly/etiology/pathology ; Tomography, X-Ray Computed

OBJECTIVETo understand the clinical feature and natural course of polycythemia vera (PV).

METHODSThe clinical symptoms, signs, laboratory examination and prognosis of 185 patients with PV were analysed.

RESULTSThere are 122 males and 63 females. The mean age was (52.7 +/- 14.1) years. The mean hemoglobin level was (208.3 +/- 21.2) g/L. Pancytosis was displayed in 74 (40%) cases, excess of red blood cells in 33 (17.8%), excess of red blood cells and granulocytes in 67 (36.2%) and excess of red blood cell and platelets in 11 (5.9%). Splenomegaly was found in 123 (66.5%) patients and hepatomegaly in 30 (16.2%). Quantitative assess of serum Epo was done in 25 patients. The level was low in 16 (64.2%) and normal in 9 (36.0%). Hematopoietic progenitor culture yields was elevated in 11 patients, endogenous erythroid colonies (EEC) formation was found in 10 cases (90.9%). Eighty two patients (44.3%) had 101 attacks of vascular thrombotic incidents, 7 patients developed myelofibrosis (MF). Secondary cancer occurred in 1 patient. Two patients died of thrombosis.

CONCLUSIONPV is an elderly adult myeloproliferative disease with a high frequency of thrombosis. EEC can be found out in PV patients. The serum Epo level is not increased in PV patients. The main sequelae of PV is MF.

Adult ; Aged ; Erythrocyte Count ; Female ; Hemoglobins ; metabolism ; Hepatomegaly ; etiology ; Humans ; Leukocyte Count ; Male ; Middle Aged ; Polycythemia Vera ; blood ; complications ; pathology ; Primary Myelofibrosis ; etiology ; Splenomegaly ; etiology ; Thrombosis ; etiology

Amyloid ; metabolism ; Amyloidosis ; complications ; diagnosis ; pathology ; Biopsy, Fine-Needle ; Congo Red ; Female ; Hepatomegaly ; etiology ; Humans ; Liver ; metabolism ; pathology ; Liver Diseases ; diagnosis ; etiology ; pathology ; Liver Function Tests ; Middle Aged ; Tomography, X-Ray Computed

Hepatitis A is generally known as a mild, self-limiting disease of the liver, but in rare instances it can progress to fulminant hepatitis, which may require liver transplantation for recovery. Such cases are known to be related to old age and underlying liver disease. We report four cases of hepatitis A in which patients presented with fulminant hepatitis and acute renal failure and underwent liver transplantation. The following common features were observed in our cases: (1) occurrence in relatively old age (> or =39 years old), (2) association with acute renal failure, (3) presence of hepatomegaly, and (4) microscopic features of submassive hepatic necrosis.
Adult ; Age Factors ; Female ; Hepatitis/complications/*diagnosis/therapy ; Hepatitis A/complications/*diagnosis ; Hepatomegaly/diagnosis/etiology ; Humans ; Kidney Failure, Acute/complications/*diagnosis ; Liver/pathology ; Liver Cirrhosis/diagnosis/etiology ; *Liver Transplantation ; Male ; Tomography, X-Ray Computed

Macrophage activation syndrome (MAS) is a rare and potentially fatal complication of rheumatic disorders in children. We describe a 13-month-old boy in whom MAS developed as a complication of systemic juvenile rheumatoid arthritis (S-JRA). He suffered from fever and generalized rash followed by multiple joints swelling for four months before admission. Physical examination revealed cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings were: abnormal liver enzymes, increased triglyceride and ferritin levels, coagulopathies resembling disseminated intravascular coagulation, anemia and thrombocytopenia. Hyperplasia of hemophagocytic macrophages was remarkable in his bone marrow. Methylprednisolone and cyclosporin therapy resulted in clinical and laboratory improvements. This is the third case of MAS associated with S-JRA in Koreans, and the first one, in which hemophagocytic macrophages were proven in bone marrow.
Alanine Transaminase/metabolism ; Alkaline Phosphatase/metabolism ; Antigens, CD/blood ; Antigens, Differentiation, Myelomonocytic/blood ; Arthritis, Juvenile Rheumatoid/blood/*complications/pathology ; Aspartate Aminotransferases/metabolism ; Blood Cell Count ; Hepatomegaly/*etiology/pathology ; Humans ; Infant ; Liver/enzymology/pathology ; *Macrophage Activation ; Male ; Partial Thromboplastin Time ; Prothrombin Time ; Splenomegaly/*etiology/pathology ; Syndrome ; gamma-Glutamyltransferase/metabolism