Ascites revealing peritoneal and hepatic extramedullary hematopoiesis with peliosis in agnogenic myeloid metaplasia: case report and review of the literature.

A 61-year-old man presented with ascites in the course of agnogenic myeloid metaplasia (AMM). Ascitic fluid was exudative and contained mature and immature leukocytes, erythroid cells, and megakaryocytes as observed on a bone marrow smear. Peritoneal biopsy showed myeloid metaplasia, and liver biopsy revealed intrasinusoidal myeloid metaplasia and peliosis. Ascites cleared after abdominal radiotherapy but treatment resulted in transient aplasia. Subsequently, portal hypertension was demonstrated by hepatic transjugular catheterization. Complications of splenomegaly led to splenectomy and splenorenal shunt followed by fatal acute hepatitis and septic shock. A review of the literature and an analysis of mechanisms of ascites occurring in AMM, especially peritoneal implants of myeloid tissue and occurrence of peliosis in myeloproliferative disorders, are presented.

[Multifocal cryptogenetic stenosing enteritis: an autonomous entity?]. / L'entérite sténosante multifocale cryptogénétique: une entité autonome?

Multifocal idiopathic stenosing enteritis has been described as a relapsing disease affecting the small bowel. The main anatomical characteristics are multiple non-specific inflammatory strictures. Six cases (4 men, 2 women) presenting this disorder were followed up for 3 to 22 years. Five patients were young. Iterative obstruction or chronic diarrhea and loss of weight were the outstanding clinical features. Hyposideremic anemia and protein-losing enteropathy were common. The anatomical substrate was numerous (2-19) short annular strictures situated in the ileum (75 p. 100). These strictures presented shallow ulcerations and underlying fibrosis which never extended beyond the submucosa. The small bowel wall was otherwise normal and did not show mucosal or vascular abnormalities at microscopic examination. In case of relapse, strictures developed distal to or in close contact with previous suture lines. Cultures for pathogenic bacteria were constantly negative. The natural history of these 6 cases and of 7 other previously published cases suggests that this disease is an autonomous disorder which must be differentiated from other inflammatory bowel disease entities such as Crohn's disease or tuberculosis.

[Hypergastrin and hyperprolactin syndrome caused by mucinous cystadenoma of the ovary]. / Syndrome d'hypergastrinie et d'hyperprolactinie dû à un cystadénome mucineux de l'ovaire.

We report a case of a clinically and biologically typical hypergastrinemia syndrome due to ovarian mucinous cystadenoma. When examined under histoimmunofluorescence, this "border-line" tumor was shown to contain a polymorphous endocrine cell proliferation, composed primarily of G cells and, to some degree, of D cells. Electron microscopic study further evidenced a small amount of prolactin containing cells, which coincided with elevated prolactin-levels in blood. Ovariectomy combined with vagotomy and pyloroplasty resulted in a prompt disappearance of both clinical and biological abnormalities. Ovarian mucinous cystadenomas have been known for a long time to contain endocrine cells, but only five cases, including the present one, have been described with fully developed endocrine expression. We suggest that this particular condition could be more frequent than generally admitted, and could justify systematic screening for mucinous cystadenoma in the case of peptide hormone dysfunction.

[Acute diarrhea in adults in a Parisian district. Clinical, bacteriologic endoscopic and histologic aspects. Study of 52 cases]. / La diarrhée aiguë de l'adulte en région parisienne. Aspects cliniques, bactériologiques, endoscopiques et histologiques. Etude de 52 cas.

From 1985 through 1988, 52 patients aged 16 to 85 years and referred for acute diarrhea underwent routine clinical, microbiological, endoscopic and histopathological examinations. Enteropathogens were isolated in 50 percent of patients, mostly from stool samples rather than from biopsy samples, though results were sometimes dissociated. Significantly lower digestive endoscopic abnormalities were seen in 60 percent of patients. Upper gastrointestinal endoscopy was not contributive. Rectal and colonic biopsies showed histological abnormalities in nearly all cases half of which were polymorphonuclear infiltrates and crypt abscesses. Glandular distortion was not found. An analysis of clinical, endoscopic and histological data showed that major abnormalities were mostly, but not constantly, related to infections due to enteroinvasive bacteria. As in other studies, a high rate of negative stool cultures was observed. Based on these results, we suggest to perform either further and more sophisticated microbiological investigations, or to conduct a routine search for viral infections whose incidence, among adult patients with acute diarrhea, is actually unknown.

[Hepatic porphyria: diagnostic and therapeutic strategies]. / Porphyries hépatiques: stratégies diagnostiques et thérapeutiques.

Acute hepatic porphyrias are genetic diseases of heme synthesis with severe prognosis due to strong or acute abdominal pain and neurological complications. Clinical evolution is characterized by acute attacks frequently induced by either forbidden drugs, or infections, alcohol intake or often unknown factors. Modern treatment is perfusion of hematin, which is a stable form of heme. Hematin will induce again delta-aminolévulinic (ALA)-synthase synthesis repression. Its tolerance is perfect whereas clinical and biochemical efficiency is absolute in our experience, if initiated very early. This drug is now considered as the treatment of acute intermittent porphyria crises.

[Detection of chronic lead poisoning. Value of the assay of erythrocyte protoporphyrin]. / Dépistage du saturnisme chronique. Intérêt du dosage de la protoporphyrine érythrocytaire.

The most common screening tests for lead poisoning are reviewed. Stippled red cells are inconstant and appear too late. Blood lead concentrations are difficult to measure accurately and often are uncorrelated with body lead stores. Excess of delta aminolevulinic acid and coproporphyrin in urine does not appear early enough. A marked increase of erythrocyte protoporphyrin level practically clinches the diagnosis, later confirmed by increase of lead in urine after an EDTA lead-mobilization test. Measurements of erythrocyte protoporphyrin levels are easy to perform, more sensitive, more precocious and cheaper than the other methods, but their relative lack of specificity must be borne in mind.

[Hepatic involvement in the acquired immunodeficiency syndrome. Study of 20 cases]. / Atteintes hépatiques au cours du syndrome d'immunodépression acquise. Etude de 20 cas.

Liver biopsies were systematically performed in 20 patients with evidence of an acquired immunodeficiency syndrome (18 with opportunistic infections, 9 with Kaposi's sarcoma). Hepatitis, related to hepatitis-B virus in 3 cases and to cytomegalovirus in 3 cases, was present in 6 of them. All patients had at least one of the three following lesions: non-specific portal inflammatory infiltration (6 cases), granulomatous lesions (12 cases), vascular abnormalities (12 cases). Among hepatic granulomatous lesions: 3 were due to acid-fast bacilli and 1 to Cryptococcus. Sinusoidal abnormality included proliferation of Küpffer cells (9 cases), sinusoidal dilatation (2 cases), peliosis (4 cases) and Kaposi's sarcoma (1 case). A relationship between these abnormalities cannot be excluded.

[Acute attacks of hepatic porphyria. Treatment with hematin. 5 cases]. / Crises aiguës de porphyries hépatiques. Traitement par l'hématine. Cinq observations.

The effectiveness of 2 hematins administered by intravenous infusion was compared in acute intermittent porphyria. Judging from subjective symptoms (abdominal pain), clinical improvement was complete and constant. There was a rapid decrease in urinary excretion of porphyrins precursors, with a clearer response of delta-aminolevulinic acid than of porphobilinogen. Urine levels of uroporphyrin often returned to normal after two infusions. The drugs were very well tolerated. Provided it is administered early, before neurological complications develop, treatment with hematin completely relieves abdominal symptoms and suppresses most of the biochemical changes associated with hepatic porphyria.