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Thrombotic microangiopathies may be initiated by a number of antecedent events. When presented with postpartum hemorrhage and unexplained thrombocytopenia, it is prudent to consider microangiopathic hemolytic anemia in the differential diagnosis. A 25-year-old woman, gravida 2, para 1, had an uncomplicated repeat Cesarean delivery at 38 weeks' gestation. She subsequently had an exploratory laparotomy for hemoperitoneum resulting from a left uterine artery laceration. On postoperative day 3, she developed thrombotic chrombocytopenic purpura-hemolytic uremic syndrome and was treated with plasma exchange therapy and dialysis. It is critical that clinicians consider this potentially fatal disease in the differential diagnosis when hemorrhagic shock is associated with unexplained thrombocytopenia, so that appropriate and early treatment may lead to a favorable outcome.

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The association of Hodgkin disease with monoclonal gammopathy has rarely been reported. We present a case of a 48-year-old woman with a history of autoimmune hemolytic anemia and Graves disease who presented with hepatosplenomegaly and a gamma-heavy-chain paraprotein. Histopathology of lymph node and bone marrow revealed nodular lymphocyte-predominance Hodgkin disease, while examination of the spleen revealed plasmacytosis consistent with gamma-heavy-chain disease. Following splenectomy, the patient has remained in complete remission for both conditions with no further treatment. To our knowledge, this is the first report of a patient with both gamma-heavy-chain disease and nodular lymphocyte-predominance Hodgkin disease. Given recent data indicating the B-cell nature of this form of Hodgkin disease, the authors propose that in this unique case there may be a clonal relationship between these 2 concurrent B-cell lymphoproliferative processes.

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The objective of this study was to determine whether the known myocardial degeneration in TTP is due to apoptosis. In TTP the heart is often involved, including the cardiac conduction system. Despite many platelet occlusions of small coronary arteries, there is little myocardial necrosis. Why the intermittent clinical episodes begin or end is unknown. Six hearts of patients dying with TTP were examined with routine and immunohistochemical stains. In addition to ventricular and atrial myocardium we examined the cardiac conduction system and coronary chemoreceptor. Numerous small coronary arteries were occluded with platelet thrombi in all these sites, including especially the sinus node, AV node and His bundle. The myocardial degeneration we found was conspicuously devoid of inflammation and the myocytes were relatively intact. These characteristics combined with TUNEL-positivity in the degenerating cells are typical of apoptosis. The focal degeneration in TTP is primarily apoptotic. Because circulating serotonin is carried by platelets and is released during aggregation, and because serotonin can cause a powerful cardiogenic hypertensive chemoreflex, we suggest that such a response may dislodge early platelet aggregations. Lessons from TTP may have special relevance for better understanding of myocardial reperfusion problems associated with angioplasty, thrombolysis and ischemic preconditioning.

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An infectious episode is the presenting manifestation of hairy cell leukemia (HCL) in approximately 30% of cases. Most often this is bacterial and only rare cases of opportunistic fungal infection are described. We report a patient who presented with sporotrichal involvement of multiple cutaneous sites and lymph nodes. The lesions resolved following antifungal therapy, but persisting pancytopenia and splenomegaly necessitated further hematological evaluation. A diagnosis of HCL was suspected based on morphologically characteristic hairy cells in the peripheral blood that contained tartrate resistant acid phosphatase. A bone marrow biopsy specimen had a normocellular marrow with an increase in interstitial lymphoid cells that stained with L26, MB2, and LN2 antibodies. On flow cytometry these cells were positive for the leukocyte common antigen, B cell markers, and the CD11c antigen confirming the diagnosis of HCL. We believe that this is the first report of sporotrichosis infection as a presenting manifestation of HCL.

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An inherited deficiency of protein C, a recognized hypercoagulable state, may cause a clinically significant deep venous thrombosis. Only some persons with a deficiency of protein C experience thrombosis, and almost always the thrombotic event occurs in the venous circulation. Warfarin-induced skin necrosis, a rare event observed in some patients soon after treatment with warfarin is begun, is believed to be another manifestation of this deficiency. We describe a young woman whose basal functional and antigenic levels of protein C were about 45% and who experienced both deep venous thrombosis and warfarin-induced skin necrosis in a clinically severe course. Evidence for lupus anticoagulants was present, with prolonged activated partial thromboplastin time that was corrected when lysed platelets were added, prolonged Russell's viper venom time, anticardiolipin antibodies, and other laboratory evidence. Lupus anticoagulants are associated also with a significant incidence of thrombosis, including arterial thrombosis, and this patient developed concurrently arterial thrombosis. The combined effects of protein C deficiency and lupus anticoagulants, exacerbated by other potentially thrombogenic conditions, are believed responsible for the severe thrombotic events experienced by this patient.

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Hemophilia is an inherited coagulation disease that affects approximately 1 in 5,000 to 10,000 males worldwide. Chronic joint disease and other long-term complications of recurrent bleeding persist in patients with hemophilia despite improved and more available clotting protein concentrates. The best care can be provided to patients who are followed regularly in specialized treatment centers. Services of every "comprehensive" hemophilia treatment center (HTC) have expanded since previous treatment with clotting factor concentrates infected many hemophilics with the human immunodeficiency virus (HIV). Each HTC offers therapeutic, educational, and counseling expertise in care for the complications of HIV. A nationwide network of specialists now provides care for patients with hemophilia and related congenital abnormalities. In Region VI (Texas, Oklahoma, and Arkansas), the treatment centers and their affiliates provide medical, psychosocial, orthopedic/physical therapy, dental, and case management services. Extramural funded research programs provide care and laboratory testing at no cost to individual subjects.

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A coagulopathy due to vitamin K deficiency was discovered in 42 hospitalized patients, most of whom had been misdiagnosed as having disseminated intravascular coagulation. Factors contributing to vitamin deficiency included inadequate diet, malabsorption, failure of physicians to prescribe vitamin K supplements, antibiotic therapy, renal insufficiency, hepatic dysfunction, recent major surgery, and possibly pregnancy. Sixteen patients (34%) bled sufficiently to need red blood cell transfusions and ten patients (24%) ultimately died. Of 18 patients who also had thrombocytopenia, three did have disseminated intravascular coagulation. The deficiency, a contributor to morbidity and mortality, can be prevented by prophylactic administration of vitamin K to severely ill patients who are eating inadequately and receiving antibiotics.

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A man with lupus anticoagulant and a prothrombin deficiency was studied before and after cessation of treatment with phenytoin. Multiple abnormal laboratory values of the following partially or completely resolved after the patient's therapy was discontinued: tissue thromboplastin inhibition ratio, prothrombin time, activated partial thromboplastin time, anticardiolipin antibodies, and quantitative measures and abnormal pattern on crossed immunoelectrophoresis of prothrombin. This patient represented an example of a concurrent drug-induced prothrombin deficiency and a lupus anticoagulant.

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Auer rod-like inclusions (ARLI) can be seen in B cell malignancies but are very rare in prolymphocytic leukemia (PLL). Prolymphocytes with ARLI from a patient with PLL were examined using cytochemical and immunochemical techniques and transmission electronmicroscopy (EM). The lymphoblastic cells gave a positive reaction with antibodies to the gamma heavy chains and kappa light chain. On EM the ARLI were 1-2 micron long, consisting of election-dense crystalline matrix, with a periodicity of 60 A and contained within a single unit membrane. The production of IgG by PLL cells with ARLI has not been previously reported. It is emphasized that in PLL the lymphoblasts can have inclusions resembling Auer rods. Cytochemical and immunochemical studies should be done to avoid a misdiagnosis of acute nonlymphoblastic leukemia.

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Dia occurs with a frequency of 8.2 to 14.7 percent in Mexican-Americans living in three Texas communities. Of 4225 blood samples tested, only six (0.14%) were Di(a+b-), and none of 2540 samples was Di(a-b-). In other studies, 1 of 525 random white blood donors was Di(a+), and Dib was well developed in 100 random umbilical cord blood samples.

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A 52-year-old gravida 1, para 1 woman with M- red cells experienced a delayed hemolytic transfusion reaction and exhibited an anti-M antibody following the infusion of four units of M+ red cells. Measurements of erythrocyte survival using 51Cr-labeled donor M+ and M- red cells and in vitro studies of monocyte-macrophage phagocytosis of sensitized reagent red cells implicate anti-M in the pathogenesis of hemolysis.

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While receiving estrogen therapy, three women with classic type I von Willebrand's disease showed significantly less bleeding and improved hemostasis. One took estrogens to prevent menopausal symptoms, and two used oral contraceptives. These women underwent major surgery without excessive bleeding and without requiring cryoprecipitate or other blood components. Prior to estrogen therapy, each woman had received cryoprecipitate or fresh frozen plasma to stop bleeding from dental extractions or other surgical procedures. Furthermore, two other women with type I von Willebrand's disease also exhibited improved hemostasis while taking oral contraceptives. These experiences suggest a short course of estrogen therapy may effectively prepare some women with von Willebrand's disease for elective surgery.

PIP: Von Willebrand's disease causes spontaneous bleeding from mucocutaneous surfaces and excessive blood loss after surgery. Some women with the disease have less spontaneous hemorrhage and show improved coagulation while taking oral contraceptives (OCs) or while pregnant; estrogens are believed responsible for these improvements. 3 women are described who had previously received infusions of either fresh frozen plasma or cryoprecipitate to stop postoperative hemorrhage, and exhibited prolonged bleeding times, abnormal platelet retention results, and decreased plasma levels of factor VIII-related coagulant, factor VIII-related antigen, and factor VIII-related von Willebrand factor. However while taking estrogens they experienced cessation of spontaneous bleeding episodes and exhibited normal coagulation test results; all observed less epistaxis and spontaneous bruising during pregnancy. Continuing estrogen therapy, each woman underwent major surgery with no abnormal bleeding an no blood component therapy. 2 other women with type 1 von Willebrand's disease also exhibited improved hemostasis while taking OCs. It is unclear how estrogens stimulate synthesis of the various factor VIII components and for certain women with von Willebrand's disease certain estrogens may improve hemostasis more effectively than others. Estrogens might obviate the need for cryoprecipitate or fresh frozen plasma to assure hemostasis, thereby eliminating the threat of transfusion-induced hepatitis and reducing surgical costs.

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In September, 1979, the Transfusion Committee at the University of Texas Medical Branch in Galveston implemented a preoperative blood ordering policy of type and screen (TS) for selected surgical procedures. During the first year in which this policy was in force, 2,301 TS requests were received, most of them for patients in the Department of Obstetrics and Gynecology. During this time, requests for cross matching declined by 12% (28,863 to 25,283) despite a 3% increase in packed red blood cell and whole blood transfusions (11,282 to 11,629). The cross match/transfusion ratio fell from 2.55 to 2.17, and outdating of blood dropped 42% (446 to 260). In the 20 months since this policy was introduced, 4,334 TS requests have been processed. Of these, 211 were changed to cross matching by the patient's physician, but only 100 patients (2.3%) actually received transfusions. No untoward reactions were observed by transfusing blood before the results of the cross matching were known. We estimate a savings to patients of $164, 920, and conclude that TS is safe and effective.

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