[Catastrophic antiphospholipid syndrome. Communication of 2 forms of presentation]. / Síndrome antifosfolipídico catastrófico. Comunicación de dos formas de presentación.

Antiphospholipid syndrome is characterized by recurrent fetal loss, arterial and venous thromboses, thrombocytopenia and circulating antiphospholipid antibodies. Few patients have a rapidly progressive, fatal outcome. We report two young patients with systemic lupus erythematosus and antiphospholipid antibodies who died after a short course of disease. Although clinical and laboratory findings differed in both patients--small vessel thromboses and microangiopathic hemolytic anemia mimicking thrombotic thrombocytopenic purpura predominated in one of the patients while small and medium size vessel thromboses without hemolysis were present in the other case--autopsy revealed widespread visceral thromboses in both of them, features consistent with a diagnosis of catastrophic antiphospholipid syndrome. This syndrome has not been reported to occur in association with Pneumocistis carinii pneumonia as we describe in one of our patients.

Case report: reactive hemophagocytic syndrome associated with disseminated strongyloidiasis.

The reactive hemophagocytic syndrome is a condition characterized by systemic proliferation of benign hemophagocytic histiocytes, fever, cytopenia, abnormal liver function, and frequently coagulopathy and hepatosplenomegaly. Its occurrence has been documented in association with viral, bacterial, fungal and parasitic infections; a wide spectrum of malignant neoplasms; some miscellaneous disorders; and phenytoin. Disseminated strongyloidiasis is reported in a patients with systemic lupus erythematosus treated with corticosteroids in whom a reactive hemophagocytic syndrome developed and who finally died. This reactive hemophagocytic syndrome is reported for the first time in strongyloidiasis and may not have been recognized in former patients.

Dysfunction of monocytes in Hodgkin's disease by excessive production of PGE-2 in long-term remission patients.

The candidacidal activity and the production of oxygen radicals by monocytes were investigated in untreated and long-term remission patients with Hodgkin's disease (HD). Both groups showed a decreased candidacidal function of monocytes with a chemiluminescence (CL) response significantly lower and delayed with respect to normal controls. Indomethacin at 1 microgram/ml corrected the monocyte deficiency increasing the CL response to normal values and normalizing the kinetics in the untreated patients. However, in patients in remission, the peak was delayed and followed by a significant increase in the production of oxygen radicals compared with untreated patients. A direct linear correlation was found between the percentages of lysed Candida and maximum CL peak of stimulated monocytes. When prostaglandin E2 (PGE-2) levels, measured in supernatants of cultured mononuclear cells, were plotted against the percentages of killed Candida, an inverse linear correlation was found. Therefore, monocytes from HD patients have a dysfunction in the generation of oxygen radicals and a decreased candidacidal activity associated with excessive production of PGE-2. Indomethacin can correct the oxidative metabolism in the untreated patients while in apparently "cured" patients the disorder persists.

Normalization of monocyte candidacidal deficiency by cyclooxygenase inhibitors in Hodgkin's disease.

In a previous work, the authors found that the peripheral blood monocytes from patients with Hodgkin's disease (HD) had depressed lytic capability to kill Candida pseudotropicalis and depressed phagocytic function. The aim of this study was to evaluate if cyclooxygenase inhibitors could correct the defective macrophage functions. Fifteen untreated patients with HD and 10 normal subjects were studied. The incubation of the cells from the patients with HD with indomethacin (IM) at 1, 3, and 10 micrograms/ml and with acetylsalicylic acid (ASA) at 20 micrograms/ml increased their previously deficient ability to kill C. pseudotropicalis, reaching values close to those of normal subjects. The oral administration of ASA during 1 week also corrected the monocyte lytic deficiency in the patients' group. Neither the in vitro nor the in vivo treatment with these cyclooxygenase inhibitors had any significant effect on normal subjects' monocytes' lytic function. The drugs did not improve the impaired phagocytic function in patients with HD. These results indicate that the failure of the lytic activity of the monocytes in HD could be associated to an excessive production of PGE2, and the oral administration of inhibitors of the cyclooxygenase activity can correct such abnormality whereas the phagocytic dysfunction is not reverted by them.

Granulopoyesis in vitro para el diagnostico y tratamiento de una anemia aplastica de probable origen inmunologico. / In vitro granulopoiesis for the diagnosis and treatment of aplastic anemia probably of immunologic origin

Se presenta el caso de un paciente de 23 anos de edad, sexo masculino, con anemia aplastica idiopatica de 3 meses de evolucion y manifestaciones hemorragicas e infecciosas, que no habia mejorado con androgenoterapia. Se le realiza cultivo de medula osea y estudio citogenetico que demuestran la existencia de un mecanismo inhibidor de la granulopoyesis, reversible in vitro con GAL y metilprednisona. La administracion de corticoides por via oral lleva a la remision del cuadro hematologico en corto plazo. La correlacion entre el estudio in vitro y la respuesta in vivo a la corticoterapia confirma la importancia de dilucidar los diferentes mecanismos responsables de la aplasia medular donde no siempre el defecto se encuentra en la celula madre

Consumption coagulopathy in acute renal failure induced by hypolipotropic diets.

Weanling male rats fed on a hypolipotropic diet develop acute renal failure whose morphological features vary from focal tubular necrosis to cortical necrosis. We have sequentially studied the hemostatic mechanism in correlation with the morphology of various tissues, mainly renal and hepatic, in choline-deficient rats as well as in three control groups. No important changes were observed in the hemostatic mechanisms before the development of tubular necrosis. Along with tubular necrosis a consumption coagulopathy was found, evidenced mainly by a decrease in the activity of factors V and VIII as well as a prolongation in PTTK and Quick's time and a decrease in platelets. Fibrin degradation products were found in serum and urine and soluble fibrin monomer complexes in the former. Following tubular necrosis thrombi were found in the renal microvasculature. It is possible to speculate that the tubular necrosis induced by choline deficiency could produce an activation of the coagulation system which in turn would lead to thrombosis of the renal microcirculation and cortical necrosis.