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Unexplained Fever In Hematologic Disorders Section 1. Benign Hematologic Disorders
Published in Benedict Isaac, Serge Kernbaum, Michael Burke, Unexplained Fever, 2019
Nonthrombocytopenic purpura can be seen in cryoglobulinemia, usually of the “mixed” type. The condition can be primary, or secondary to various collagen-vascular diseases or to infectious disease. Exposure to cold causes the precipitation of the cryoglobulins and can provoke Raynaud’s phenomenon, various skin lesions, and the hyperviscosity syndrome.
Primary Hyperfibrino(Geno)Lytic Syndromes
Published in Genesio Murano, Rodger L. Bick, Basic Concepts of Hemostasis and Thrombosis, 2019
Rodger L. Bick, Genesio Murano
Little attention has been devoted to vascular defects during CPB. Recently, a syndrome of mild to moderate nonthrombocytopenic purpura accompanied by splenomegaly and atypical lymphocytosis following CPB has been reported by Behrendt and co-workers.43 In this series, purpura was benign, self-limiting, and frequently only manifest after discharge from the hospital. Only one patient of seven suffered complications (glomerulonephritis of the type often seen with Henoch-Schonlein purpura) following development of purpura.
Syndromes Associated with Hyperfibrino(GENO)Lysis
Published in Rodger L. Bick, Disseminated Intravascular Coagulation and Related Syndromes, 2019
Little attention has been devoted to vascular defects during bypass surgery. Recently a syndrome of mild to moderate nonthrombocytopenic purpura accompanied by splenomegaly and atypical lymphocytosis following CPB has been reported by Behrendt and co-workers.72 In this series of patients purpura was benign, self-limiting, and frequently only manifest after discharge from the hospital. Only one patient, of seven, suffered complications (glomerulonephritis of the type often seen with Henoch-Schonlein purpura) following the development of purpura. In addition, a case of fatal purpura fulminans was reported following extracorporeal circulation for coronary artery bypass surgery.73 Massive purpura developed on the third postoperative day and was followed by progressive renal failure. High doses of steroids and low molecular weight dextran afforded no improvement and the patient died of renal failure on the 18th postoperative day. These two reports suggest the possibility of an inflammatory vasculitis to be associated with some instances of cardiac surgery. The most benign forms are represented by purpura simplex. Rarely, purpura fulminans may also occur. Aside from these two reports, no other mention of vascular defects associated with CPB can be found in the literature.
IgA negative immunofluorescence in diagnoses of adult-onset Henoch-Schönlein purpura
Published in Baylor University Medical Center Proceedings, 2020
Pooja Poudel, Steven H. Adams, Kanish Mirchia, Hanish Jain, Ambika Eranki
In conclusion, although HSP is largely seen in children, it is a disease to be considered in adults presenting with nonthrombocytopenic purpura with arthralgias, abdominal complaints, and/or hematuria. These cases highlight that although HSP is classically associated with deposition of IgA within vessel walls, the disease can be diagnosed even with a negative IgA immunofluorescence biopsy based upon clinical findings alone. If a biopsy is desired for extra confirmation of clinical suspicion, it may be more useful to perform the biopsy closer to the onset of symptoms.