[Granulomatous hepatitis and myelitis: an unusual manifestation of extrapulmonary sarcoidosis].
Sarcoidosis is a chronic multisystem disorder of unknown cause characterized by the presence of noncaseating epitheloid granulomas and derangement of the normal skin architecture. Though an array of organs may be affected by the disease the most common site of affection is the lung. An extrathoratic manifestation is rare. We describe a 66-year-old patient who was admitted to our hospital because of weight loss and hepatomegaly. A thorough examination revealed the diagnosis of a granulomatous hepatitis characterized by a markedly elevated alkaline phosphatase concentration of 1,490 U/I. A drug-induced hepatitis could be excluded and no evidence was found for the existence of a bacterial or viral infection or an autoimmune disorder. An ERCP revealed a normal common bile duct and normally branching small intrahepatic ducts. The patient was discharged with the diagnosis of a biliary cirrhosis. Half a year later the patient was readmitted to the hospital because of severe intestinal bleeding due to pancytopenia. A bone marrow biopsy showed infiltration of the marrow by granulomas. A histiocytosis X could be ruled out. The diagnosis of an extrathoracic sarcoidosis was assumed and a therapy with prednisone was started. Within six weeks the blood count normalized. After 18 months the serum alkaline phospatase concentration also normalized and no granulomas were found in the bone marrow. The case demonstrates that pancytopenia in sarcoidosis is not due to bone marrow failure.
Trenn
G
G
Krankenhaus Maria Hilf, Med. Klinik 1, Mönchengladbach.
Gabbert
H E
HE
Schmitt-Gräff
A
A
Reis
H E
HE
D000368:Aged; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D006505:Hepatitis / Q000473:pathology*; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D010019:Osteomyelitis / Q000473:pathology*; D010198:Pancytopenia / Q000473:pathology; D012507:Sarcoidosis / Q000473:pathology*