[Wilson's disease and secondary copper hemochromatoses in hematological practice].
OBJECTIVE
To characterize clinical, diagnostic and therapeutic syndromes of copper overloading in patients with hepatic lesion in combination with hemopoietic disorder.


METHODS
Treatment results and diagnostic findings are presented for patients with clinical picture of liver cirrhosis, cytopenia and copper overloading. The examination included standard clinical and specific tests, morphological investigation of the bone marrow, copper metabolism in dynamics.


RESULTS
A case of a patient is reported in whom Wilson's disease presented in debut with a picture of decompensated liver cirrhosis and immune thrombocytopania complicated by recurrent hemorrhagic syndrome. D-penicillomine treatment initiated ex juvantibus allowed verification of the diagnosis of Wilson's disease and achievement of marked clinical response. In another case laboratory signs of copper overloading were revealed in a patient with liver cirrhosis of viral etiology (HBsAg+) and deep cytopenia associated with uneffective hemopoiesis. Chelator therapy with D-penicillamine regressed cytopenic syndrome and improved functional capacity of the liver.


CONCLUSIONS
Primary or secondary nature of copper overloading in patients with hepatic cirrhosis and critical cytopenia, pathogenesis of cytopenic syndrome, practical significance of copper hemochromatoses diagnosis are discussed.
Vorob'ev
A I
AI
Lukina
E A
EA
Sysoeva
E P
EP
Levina
A A
AA
Solov'eva
T I
TI
D000328:Adult; D002614:Chelating Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D003300:Copper / Q000097:blood / Q000652:urine; D003937:Diagnosis, Differential; D004334:Drug Administration Schedule; D006432:Hemochromatosis / Q000175:diagnosis / Q000188:drug therapy / Q000378:metabolism; D006527:Hepatolenticular Degeneration / Q000175:diagnosis / Q000188:drug therapy / Q000378:metabolism; D006801:Humans; D008103:Liver Cirrhosis / Q000175:diagnosis; D008297:Male; D010396:Penicillamine / Q000008:administration & dosage / Q000627:therapeutic use*; D016896:Treatment Outcome
D002614:Chelating Agents; D003300:Copper; D010396:Penicillamine
Bolezn' Vil'sona i vtorichnye mednye gemokhromatozy v praktike gematologa.