B Sattler

Recurrent episodes of acute hepatitis associated with LKM-1 (cytochrome P450 2D6) antibodies in identical twin brothers

BACKGROUND/AIMS Liver/kidney microsomal antibodies have been noted in liver disease of different etiology, e.g. in autoimmune hepatitis, chronic hepatitis C and D virus infection and in drug-induced liver disease. Unlike these, acute hepatitis of unknown etiology associated with high-titer liver/kidney microsomal-1 antibodies (cytochrome P450 2D6) is reported in identical twin brothers. METHODS Patients were studied using clinical, biochemical, serological and immunological methods, as well as liver biopsy. RESULTS The acute icteric episodes were followed by spontaneous remission with complete normalization of liver function tests and liver histology. During the acute phase, serum titer for liver/kidney microsomal-1 antibodies (detected by indirect immunofluorescence, ELISA and Western blot analysis) was exceedingly high and decreased gradually thereafter. Hepatitis C and D virus infection were excluded by repeated serological testing; exposure to drugs or chemicals was not evident. Concomitant autoimmune disease was not detectable. HLA typing for class 1 and 2 antigens was positive for the HLA haplotype DQ2, but negative for HLA B4, B8, DR3 and DR4. CONCLUSIONS The present observations might suggest a hitherto unreported form of acute hepatitis of unknown etiology, distinct from other liver diseases in which liver/kidney microsomal antibodies have been described so far.

Undifferenziertes kleinzelliges Hepatoblastom

ZusammenfassungUndifferenzierte kleinzellige Hepatoblastome (HB) zählen zu den seltenen malignen Tumoren der Leber im Kindesalter. Da der Tumor in der Regel kein α-Fetoprotein exprimiert, ist der Nachweis von Zytokeratin 8, 18 und 19 sowie Vimentin und Aktin diagnostisch wegweisend. Als Ausgangszelle wird eine pluripotente, wohl entodermale Stammzelle vermutet. In der Gruppe der klein-, rund- und blauzelligen malignen Tumoren des Kindesalters bietet diese Variante des HB differenzialdiagnostische Schwierigkeiten. Wir berichten über ein undifferenziertes kleinzelliges HB eines 15 Monate alten weiblichen Kleinkindes mit Agenesie der rechten Niere. Als morphologische Besonderheit des Tumors werden disseminierte histiozytäre Riesenzellen beschrieben.AbstractUndifferentiated small-cell hepatoblastoma (HB) is a rare malignant tumor of childhood. The cell of origin is supposed to be a pluripotential, probably entodermal, stem-cell. Differential diagnosis of this type of HB is difficult among the group of small round and blue cell malignant tumors of children. The immunohistochemically determined coexpression of cytokeratin 8, 18, and 19 and of vimentin and actin, regularly in the absence of α-fetoprotein expression may be diagnostically helpful. We present the case of an undifferentiated small-cell HB of a 15-month-old girl with agenesis of the right kidney. As morphological peculiarity the tumor presented disseminated histiocytic giant cells.

Undifferentiated small cell hepatoblastoma with a chromosomal translocation t(22;22)(q11;q13)

granulomas in hairy cell leukaemia following 2-chlorodeoxyadenosine therapy. Histopathology 1994; 24; 271–273. 2. Schmidt HH, Hofler G, Beham-Schmid CH, Sill H, Linkesch W. Bone marrow granulomas in hairy cell leukaemia. Histopathology 1996; 29; 291–293. 3. Bendix-Hansen K, Bayer Kristensen I. Granulomas of spleen and liver in hairy cell leukaemia. APMIS 1984; 92; 157–160. 4. Broady R, Roberts S, Hawkins T. Mycobacterium avium intracellulare complex infection following 2-chlorodeoxyadenosine therapy for hairy cell leukaemia. Leukemia Lymphoma 2000; 36; 639–642. 5. O’Brien DV, Boon AP, Boughton BJ. Bone marrow granulomas in acute myeloid leukaemia following interleukin 2 and lymphokineactivated killer cells. Histopathology 1992; 20; 271–272. 6. Coci A, Castello A, Pagnucco G et al. Bone marrow histology in patients with hairy cell leukemia (HCL) treated by human lymphoblastoid interferon. Haematologica 1987; 72; 143–149.

Gallenblasenkarzinom: Aggressive Chirurgie ja oder nein?

Einleitung: Gallenblasenkarzinome haben eine ungunstige Prognose. Eine 5-Jahres-Uberlebensrate ohne Therapie liegt bei unter 5%. Mehr als 50% der inzidentiellen Karzinome betreffen die Stadien I–II. Patienten und Methoden: 23 Patienten (14 w, 9 m), medianes Alter 60,4 (47–85) Jahre. Tl: 1, T2: 3, T3: 6 und T4: 13 Patienten. Die elektive Operation im Fruhstadium bestand aus einer erweiterten Cholezystektomie mit radikaler Lymphadenektomie. Ergebnisse und Schlusfolgerung: Die Uberlebensrate nach 6 Monaten war bei T2: 65%, T3: 80% und T4: 20%. Beim inzidentiellen Karzinom war die Prognose gunstiger, da unabhangig vom Tumorstadium die Uberlebenszeit uber 6 Monate lag. Im Fruhstadium ist eine radikale Operation nicht nur unter dem Gesichtspunkt der Radikalitat sondern auch der Palliation angezeigt.