[Recurrent spontaneous hemorrhage in a patient with light chain immunocytoma].
The 48-year-old female patient was sent to our clinic for further evaluation of a spontaneous decrease of prothrombin- and prolongation of the bleeding-time. She presented in good conditions with an enlargement of cervical lymphnodes and the history of a monoclonal plasmacyte dyscrasia. The laboratory results revealed a pronounced decrease of prothrombin-time, a prolonged activated partial thromboplastin-time, a decrease of factor VII and X activity and a light chain paraprotein. The histological examination of the bone marrow led to the diagnosis of an immunocytoma and a medullar amyloidosis. For the aim of influencing the coagulopathy the patient was treated with chemotherapy. However, she developed severe bleedings. Further haemostaseological tests presented an amyloidosis-associated decrease of factor VII and X, an acquired von Willebrands disease, an acquired thrombozytopathy and a lupus-like anticoagulans. Under substitution of factor VIII-von Willebrand-factor-complex and chemotherapeutic treatment a stabilisation over several years was achieved till the patient died due to an amyloid-associated acute pancreatitis.
Neuhaus
T
T
Medizinische Universitäts-Poliklinik Bonn. T.Neuhaus@uni-bonn.de
Pötzsch
B
B
Ko
Y
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Köhler
G
G
Brackmann
H H
HH
Vetter
H
H
D000686:Amyloidosis / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D001780:Blood Coagulation Tests; D001855:Bone Marrow Diseases / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D007146:Immunoglobulin lambda-Chains; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D008875:Middle Aged; D011517:Prothrombin Time; D012008:Recurrence
D007146:Immunoglobulin lambda-Chains