[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.

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

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.

[Use of transjugular intrahepatic portosystemic shunt (TIPS) in an 84 year-old patient during acute variceal bleeding]. / Utilizacion del shunt portosistemico intrahepatico transyugular (TIPS) en un paciente 84 años durante un episodio de hemorragia aguda por varices esofagicas.

The transjugular intrahepatic protosystemic shunt (TIPS) is a nonsurgical method with low mortality which does not interfere with subsequent liver transplantation. We describe the case of an old male patient with chronic hepatopathy who was admitted because of an episode of variceal bleeding. Due to the failure of the medical treatment and sclerotherapy, coronarian stomachic vein was embolized with a coil and a TIPS was performed during the acute episode of variceal bleeding. He evolved favorably and after a year of the procedure remains asymptomatic.

Rupture of mitral chorda tendinea following blunt chest trauma.

Acute mitral insufficiency, originated from rupture of mitral chordae tendineae secondary to nonpenetrating thoracic trauma, is an unusual condition. This diagnosis is difficult to establish because physical examination, electrocardiogram, and cardiac enzymes are neither sensitive nor specific. The diagnosis of rupture must be quickly established because this disorder may be fatal. This paper reports the case of a patient with acute mitral insufficiency secondary to a mitral valve chord rupture a week after a nonpenetrating thoracic trauma.

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.

Pulmonary hypertension in paroxysmal nocturnal hemoglobinuria.

Pulmonary arterial hypertension (PAH) and cor pulmonale were found in a patient with paroxysmal nocturnal hemoglobinuria (PNH). Autopsy revealed widespread thromboses in pulmonary microvasculature. Vascular thromboses attributed to hypercoagulability have been found in PNH in many organs, including the lungs. PAH has not been reported, however. This disease should then be considered a rare cause of PAH.