Craig S. Kitchens

Acquired Hemophilia: A Natural History Study of 16 Patients With Factor VIII Inhibitors Receiving Little or No Therapy

Rarely, a patient develops an antibody against factor VIII coagulant activity. The resultant hemorrhagic diathesis is clinically distinct from inherited hemophilia, being characterized by few hemarthroses but frequent skin and other soft-tissue hematomas. Hematuria may be troublesome. These patients represent therapeutic challenges. This study is one institutions results with 16 such patients followed up over an average of 31 months (range, four to 120 months; median, 19 months). It describes the largest group from a single institution receiving essentially no immunosuppressive agents, yet has one of the better overall results. Two patients experienced fatal hemorrhage and five patients underwent spontaneous remission. Long-term survival is not incompatible with persistence of the inhibitor. We conclude that this hemorrhagic diathesis is clinically distinct, less fatal than usually perceived, and may undergo spontaneous remission. Clearly, there is no mandate for any particular therapeutic regimen, such as immunosuppression, in the attempt to rid the patient of the antibody.

Acquired functional protein s deficiency, cerebral venous thrombosis, and coumarin skin necrosis in association with antiphospholipid syndrome: report of two cases

We report for the first time an association among lupus anticoagulant, coumarin skin necrosis, and cerebral venous thrombosis. We found that serum concentrations of free functional protein S were markedly depleted because of excessive binding of protein S to C4b binding protein in two patients at the time they experienced these unusual thromboses

Are Transfusions Overrated? Surgical Outcome of Jehovah's Witnesses

Physicians as well as their patients are quite familiar with the ever growing list of complications of transfusion. Blood is usually administered by physicians with the nearly unchallenged view that failure to transfuse would have dire consequences. Evidence supporting that view is very difficult to obtain. Although no controlled trial exists, data are collected from 16 reports of the surgical outcome of a series of patients of the Jehovahs Witness faith who were not given transfusion for operations during which transfusion is typically given. Analysis of these data supports the concept that approximately 0.5% to 1.5% of such operations are complicated by anemia resulting in death. This risk of not transfusing patients must be weighed against the cost, morbidity, and mortality that would be expected to accrue had these patients been transfused. These concepts should be employed whenever one is formulating a risk-benefit ratio for patients for whom transfusion is contemplated.

Aeromonas hydrophile bacteremia in ambulatory immunocompromised hosts

We observed seven episodes of Aeromonas hydrophila septicemia in five patients. All but one patient had an underlying disease resulting in immunosuppression. All episodes occurred in ambulatory patients whose vocation or avocation had recently caused them to contact water or fish. We hypothesize that infection with this gram-negative bacterium is acquired more frequently from the natural habitat of the organism than from nosocomial sources.

Thrombocytopenia and thrombosis in disseminated intravascular coagulation (DIC)

Disseminated intravascular coagulation (DIC) is the physiologic result of pathologic overstimulation of the coagulation system. Despite multiple triggers, a myriad of laboratory abnormalities, and a clinical presentation ranging from gross hemostatic failure to life-threatening thrombosis, or even both simultaneously, a simplified clinical approach augmented by a few readily available tests allows prompt identification of the process and elucidation of treatment opportunities. Platelet counts in DIC may be low, especially in acute sepsis-associated DIC, yet increased in malignancy-associated chronic DIC. Thrombotic risk is not a function of the platelet count, and thrombocytopenia does not protect the patient from thrombosis. The stratification of both thrombotic risk and hemorrhagic risk will be addressed.

Fatality in a case of envenomation byCrotalus adamanteus initially successfully treated with polyvalent ovine antivenom followed by recurrence of defibrinogenation syndrome

IntroductionRecurrences of clinical or laboratory manifestations of North American pit viper envenomation may happen despite control of the envenomation syndrome by prompt and adequate antivenom therapy. Recurrences of coagulopathy in victims of Eastern diamondback rattlesnake envenomation are generally regarded as benign. The vast majority suffer no actual bleeding despite florid coagulation laboratory abnormalities due to selective defibrinogenation.Case ReportWe report what we believe to be the first fatality following successful control of the envenomation syndrome following ovine antivenom treatment resulting from envenomation by a bite from the Eastern diamondback rattlesnake. This case raises the question of whether such recurrences are in fact benign, causal, or coincidental. This patient sustained significant brain hemorrhage and death ensued due to generalized cerebral edema. Defibrinogenation occurred 4 days after treatment with ovine antivenom.DiscussionCoagulation abnormalities following Eastern diamondback rattlesnake envenomation are due to selective defibrinogenation. This is separate from disseminated intravascular coagulation (DIC). Thrombin generation, thus hemostasis, are generally considered normal. This case may cause reexamination of this belief.

Partial deficiency of coagulation factor XI as a newly recognized feature of Noonan syndrome

Four patients with stigmata and cardiovascular abnormalities of Noonan syndrome were found tohave prolonged partial thromboplastin times. Coagulation evaluation disclosed that each had partial deficiency of blood coagulation factor XI (range 25 to 45% of normal; mean 35%). Factor XI deficiency has not been recognized as one of the stigmata of Noonan syndrome. Because of the hemostatic stress of cardiothoracic surgery, factor XI deficiency should be sought in patients with Noonan syndrome.

The effect of low-dose oral contraceptives on cardiorespiratory function, coagulation, and lipids in exercising young women: A preliminary report

A study was undertaken to determine whether low-dose oral contraceptive usage would negate the beneficial effect of exercise on cardiorespiratory fitness, lipid and lipoprotein levels, and coagulation. Twelve exercising women were randomly allocated to groups of either oral contraceptive users or non-oral contraceptive users. When compared with results in the control group, maximal oxygen uptake (ml/kg1 X min1) decreased significantly in the oral contraceptive users during the 6-month period of observation. This was associated with an 8% decrease in both the oxygen uptake (2.34 to 2.17 L/min) and the oxygen pulse (12.1 +/- 3.2 to 11.2 +/- 2.2 ml/beat). The serum cholesterol, triglycerides, high-density lipoprotein/cholesterol, and high-density lipoprotein subfractions 2a and 2b levels were not altered. A significant increase in plasminogen activity was found in the oral contraceptive users: values increased from a coherent time average of 3.8 +/- 0.5 U/ml at baseline to 5.7 +/- 0.7 U/ml at 6 months; values returned to baseline levels 1 month after stopping the oral contraceptives (coherent time average of 3.9 +/- 0.6 U/ml; p less than 0.0001). No other significant changes were noted in the coagulation and anticoagulation factors studied. Low-dose oral contraceptive usage is associated with a decrease in functional aerobic capacity, but it does not impinge on the hemostatic mechanism or lipid-lipoprotein metabolism.

Dermatomyositis associated with malignant melanoma. Parallel occurrence, remission, and relapse of the two processes in a patient

A young man developed metastatic melanoma and classic dermatomyositis. With surgical extirpation of the metastasis, the dermatomyositis resolved only to reccur simultaneously with further metastases. To the knowledge of the authors, this case represents the best‐documented parallel course of these two diseases.

Intravenous immunoglobulin administration in the treatment of severe chronic immune thrombocytopenic purpura

Human immunoglobulin was administered intravenously to nine adult patients having severe chronic immune thrombocytopenia purpura. The response in three patients was an increase in the platelet count to greater than 50,000/mm3, a hemostatically adequate level. Response was associated with a pretreatment platelet-associated immunoglobulin level of more than 5,000 molecules per platelet, and successful treatment resulted in a decrease in that level. In those patients with pretreatment platelet-associated immunoglobulin levels less than 5,000 molecules per platelet, there was neither a significant decrease in that level nor an increase in their platelet count. Immunoglobulin infusion may prove useful for selected patients with severe chronic immune thrombocytopenia.