Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the clinical and pathologic findings in one case of mast cell leukemia observed in a series of 60 patients with systemic mast cell disease. The leukemic variant of systemic mast cell disease is rapidly fatal (mean duration of survival, less than 6 months) in contrast to most nonleukemic cases, which follow an indolent clinical course. On the basis of our case and eight previously reported cases, mast cell leukemia is characterized by a substantial increase in atypical mast cells in the peripheral blood, diffuse infiltration with atypical mast cells in the bone marrow, a strong association with peptic ulcer disease, prominent constitutional symptoms, and hepatosplenomegaly. These cases should be distinguished from malignant mastocytosis without a substantial number of circulating atypical mast cells and also cases of acute nonlymphocytic leukemia that arise in the background of systemic mast cell disease.
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PMID:Mast cell leukemia: report of a case and review of the literature. 309 98

A radiological diagnosis of gastric volvulus (GV) was made in 11 of 576 consecutive upper gastrointestinal series at the University of Benin Teaching Hospital, Nigeria, over a two-year period. The clinical symptoms were thoracico-abdominal in three and abdominal in eight; these cases were evaluated as acute in three, acute upon chronic in two, and chronic in six. There was a significant delay in the diagnosis in all cases (except a neonate in the series), and no case was diagnosed on clinical grounds alone.THE PREDISPOSING FACTORS (EXCEPT THE CLINICAL MISDIAGNOSIS OF THE NEONATE) IN SIX OF THE SEVEN CASES THAT CAME TO SURGERY WERE: diaphragmatic hernia and perigastritis (left lung abscess, thoracic empyema), arteriomesenteric compression of the duodenum in pregnancy (peptic ulcer), splenomegaly (hepatosplenomegaly, ascites, esophageal varices), previous gastrojejunostomy (stomal ulcer, left subphrenic abscess) and two cases of intestinal malrotation with mesenteric abnormalities (small bowel obstruction in one and duodenal atresia in the other). In one idiopathic case, gastric outlet obstruction was clinically suspected prior to surgery. Thus, the putative rarity of GV in black Africans is not supported by this experience.Gastric volvulus is a clinico-radiologic entity that may present with a confusing thoracico-abdominal symptom complex. A greater awareness of the radiologic features is quintessential to an expeditious and usually successful surgical management that will avoid potentially serious complications. Negative surgical findings do not exclude GV as the underlying cause of acute abdomens necessitating emergency laparotomies.
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PMID:Volvulus of the stomach: an African series and a review. 356 Feb 44

Thirty-nine consecutive patients with acute lymphoblastic leukemia were treated with an intensive chemotherapy protocol. There were 23 males and 16 females with a median age of 37 years (range: 15-65). Eighteen patients had common ALL, seven had pre-B ALL, three early-precursor B ALL, seven T-ALL and four had aberrant expression of myeloid antigens (c-ALL in three and pre-B ALL in one). The median initial leukocyte count was 11.8 x 10(9)/l (range: 0.65-295). Cytogenetic result of the marrow was available in 16 of 39 patients (41 per cent) and showed Philadelphia positivity in six, a normal result in six and one each of t(4,11), t(1,19), hyperdiploidy and del 12p. Hepatosplenomegaly was present in about 20 per cent of the patients. L-Asparaginase-related hepatic toxicity was the commonest toxicity (48.7 per cent) during phase I of induction. Prolonged pancytopenia and hypoplastic death were common during phase II. With the use of growth factors during the neutropenic period of phase II induction, the rate of hypoplastic death was reduced from 40 per cent to 3 per cent. Common causes of treatment failure included early hypoplastic death (27.8 per cent) and leukemia relapses (50 per cent) while primary refractory leukemia, hepatic failure and perforated peptic ulcer contributed to 11.1, 5.5 and 5.5 per cent of the other deaths. A high complete remission (CR) rate (87.4 per cent) was achieved after phase I induction. The median event-free survival (EFS) was 8 months and the 3-year event-free survival was 43 per cent. This result compared favourably to the other regimens previously employed in our institution. In conclusion, satisfactory survival can be achieved with this intensive regimen. Good supportive care was however, essential to minimize toxicities.
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PMID:Improved treatment outcome in adult acute lymphoblastic leukemia using the intensive German protocol, a preliminary report. 937 68