Jack B. Alperin

Estrogens and surgery in women with von Willebrand's disease

While receiving estrogen therapy, three women with classic type I von Willebrands 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 Willebrands 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 Willebrands disease for elective surgery.

Hemoglobin sabine beta 91 (f 7) leu to pro. An unstable variant causing severe anemia with inclusion bodies.

Abstract Hemoglobin Sabine (α2β2 91 leu → pro) comprises 8 per cent of the hemoglobin of a 16-year-old Scotch-English-German girl who has suffered from hemolytic anemia since infancy. The spleen wa...

Platelet antagonists in migraine prophylaxis. A clinical trial using aspirin and dipyridamole.

SYNOPSIS

Type and screen: A safe and effective preoperative blood ordering policy with emphasis on its use in obstetrics and gynecology

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 patients 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

Anaphylactic reactions following infusion of factor VIII in a patient with classic hemophilia

164, 920, and conclude that TS is safe and effective.

IVIg for Treatment of Severe Refractory Heparin-Induced Thrombocytopenia

A 28 year old white man with hemophilia A had, for several years, been successfully maintained on a home care program utilizing self-administration of factor VIII concentrates, but one such infusion resulted in severe anaphylaxis. An immunoglobulin E (IgE)-mediated hypersensitivity response was demonstrated by (1) the release of histamine from the patients basophils in vitro upon challenge with several different lots of factor VIII concentrate; (2) immediate cutaneous response to commercial factor VIII, factor IX and cryoprecipitate; and (3) measurement of IgE antibodies against a commercial factor VIII preparation. A subsequent life-threatening hemorrhage required cautious infusion of another commercial lot of factor VIII concentrate. Despite pretreatment with antiallergic drugs and attempted desensitization, a moderately severe anaphylactic reaction was observed.

Thrombotic thrombocytopenic purpura and adult onset still's disease

BACKGROUND: Heparin‐induced thrombocytopenia (HIT) complicated by severe thrombocytopenia and thrombosis can pose significant treatment challenges. Use of alternative anticoagulants in this setting may increase bleeding risks, especially in patients who have a protracted disease course. Additional therapies are lacking in this severely affected patient population. METHODS: We describe three patients with HIT who had severe thromboembolism and prolonged thrombocytopenia refractory to standard treatment but who achieved an immediate and sustained response to IVIg therapy. The mechanism of action of IVIg was evaluated in these patients and in five additional patients with severe HIT. The impact of a common polymorphism (H/R 131) in the platelet IgG receptor Fc&ggr;RIIa on IVIg‐mediated inhibition of platelet activation was also examined. RESULTS: At levels attained in vivo, IVIg inhibits HIT antibody‐mediated platelet activation. The constant domain of IgG (Fc) but not the antigen‐binding portion (Fab) is required for this effect. Consistent with this finding, IVIg had no effect on HIT antibody binding in a solid‐phase HIT immunoassay (platelet factor 4 enzyme‐linked immunoassay). The H/R131 polymorphism in Fc&ggr;RIIa influences the susceptibility of platelets to IVIg treatment, with the HH131 genotype being most susceptible to IVIg‐mediated inhibition of antibody‐induced activation. However, at high doses of IVIg, activation of platelets of all Fc&ggr;RIIa genotypes was significantly inhibited. All three patients did well on long‐term anticoagulation therapy with direct oral anticoagulants. CONCLUSIONS: These studies suggest that IVIg treatment should be considered in patients with HIT who have severe disease that is refractory to standard therapies.

Iron deficiency anemia and papilledema - Rapid resolution with oral iron therapy

BACKGROUND Thrombotic thrombocytopenic purpura (TTP) has a high mortality rate if undiagnosed and untreated. Although recent literature supports the role of ADAMTS13 (a disintegrin-like metalloproteinase with thrombospondin type 1 repeats), the von Willebrand factor cleaving protease, in the pathogenesis of the disease, many aspects of the disease remain a mystery. Various drugs and autoimmune conditions, such as systemic lupus erythematosus and the antiphospholipid syndrome, have been observed in association with TTP. Adult onset Stills disease (AOSD) has been reported less frequently in association with TTP. PRESENTATION We report the case of a 43-year-old African American man who initially presented with fever and joint pain and was later diagnosed with TTP. He responded initially to plasma exchange, but never achieved complete remission. He eventually required splenectomy for complete resolution of symptoms of TTP, but the arthritis never resolved, resulting in several admissions for joint pain. The arthritis was eventually diagnosed as AOSD. DISCUSSION Literature review shows that the autoimmune diseases usually associated with TTP include systemic lupus erythematosus and the antiphospholipid syndrome. Eight reports of AOSD with TTP have been reported, but our case is unique in several aspects. Previous case reports have described TTP occurring in patients with known AOSD; here, we describe TTP preceding or coinciding with the onset of AOSD. Interestingly, the patients AOSD-associated arthritis responded to plasma exchange, but did not resolve after splenectomy. The coincident onset of AOSD and TTP in this patient lead us to suspect a common pathophysiologic pathway in the pathogenesis for both of these diseases.

A comparison of hemoglobin A1c in human and baboon blood.

SummaryA young female with papilledema and iron deficiency anemia is described. The papilledema was resolved rapidly after oral iron therapy was initiated.

A new ceruloplasmin variant, Cp Galveston

Cation exchange chromatography was performed on hemolysates prepared from human and baboon (Papio anubis) blood. In humans with diabetes mellitus and in baboons with pancreatectomy induced diabetes mellitus there were significant increases in Hbs A1, A1c, and A1a +A1b. The quantity of Hb A1 and Hb A1c in nondiabetic baboons was approximately one-half the quantity of Hb A1 and Hb A1c in nondiabetic humans. These differences may be explained by the differences in survival time between human and baboon red cells.