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At least 73 records · Page 4Linked to original sources
PubMed1980

[Panniculitis with cellular phagocytosis. Chronic form of histiocytic panniculitis with fever, pancytopenia, polyserositis and lethal hemorrhagic diathesis].

Two cases of what was originally called "Weber-Christian disease" were found to be a chronic, recurrent form of histiocytic panniculitis characterized by histiocytic engulfment of red and white blood cells--"cytophagocytosis." The disease was manifested by recurrent fever, subcutaneous nodules, pancytopenia, and mucosal ulcerations. Hepatosplenomegaly and enlarged lymph nodes were observed. Focal invasion of the reticuloendothelial system by acidophilic or cytophagic histiocytes could be identified. Progressive changes in liver function and hemorrhagic diathesis occurred in both patients.

Adipose Tissue↗
PubMed1999

[A patient with isolated prolongation of aPTT without hemorrhagic diathesis anamnesis: severe, hereditary factor XII deficiency].

By virtue of a severely prolonged aPTT with a normal thromboplastin time (prothrombin time) and a normal thrombin time, severe FXII deficiency has been diagnosed in a woman without a bleeding diathesis or a history of thromboembolic complications. A deficiency of a factor of the contact activation system (FXII, prekallikrein, high molecular weight kininogen) is usually diagnosed during routine coagulation tests demonstrating a prolonged aPTT. The severe and partial deficiency of FXII, of prekallikrein or high molecular weight kininogen is not associated with a bleeding tendency. In contrast, severely factor XI deficient subjects may suffer from a mild hemorrhagic diathesis, whereas FVIII deficiency (hemophilia A, autoimmune "hemophilia", von Willebrand disease) and FIX deficiency (hemophilia B) are associated with a bleeding tendency of varying severity, depending on the clotting activity of FVIII or FIX, respectively. An isolated prolongation of the aPTT due to a lupus anticoagulant, however, is frequently associated with arterial and/or venous thrombosis. Therefore, in case of a prolongation of the aPTT, its cause has to be determined.

Adult↗