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@article{1086746,
author = {Szturz, Petr and Kyclová, Jitka and Moulis, Mojmír and Navrátil, Milan and Adam, Zdeněk and Vaníček, Jiří and Mayer, Jiří},
article_location = {Praha},
article_number = {1},
keywords = {amyloidosis; hemoperitoneum; hemangiosarcoma; computed tomography; B-symptoms},
language = {eng},
issn = {0862-495X},
journal = {Klinická onkologie},
title = {Extensive AL Amyloidosis Presenting with Recurrent Liver Hemorrhage and Hemoperitoneum: Case Report and Literature Review},
volume = {26},
year = {2013}
}
TY - JOUR
ID - 1086746
AU - Szturz, Petr - Kyclová, Jitka - Moulis, Mojmír - Navrátil, Milan - Adam, Zdeněk - Vaníček, Jiří - Mayer, Jiří
PY - 2013
TI - Extensive AL Amyloidosis Presenting with Recurrent Liver Hemorrhage and Hemoperitoneum: Case Report and Literature Review
JF - Klinická onkologie
VL - 26
IS - 1
SP - 49– 52
EP - 49– 52
PB - Česká lékařská společnost J.E.Purkyně
SN - 0862495X
KW - amyloidosis
KW - hemoperitoneum
KW - hemangiosarcoma
KW - computed tomography
KW - B-symptoms
N2 - Spontaneous hepatic bleeding is a rare but potentially life-threatening complication of primary systemic amyloidosis. Although the liver is a common site of amyloid deposition, clinical presentation is usually mild or absent. Case: We report a case of a female patient, who had been repeatedly surgically revised because of liver rupture and hemoperitoneum. Initially, the computed tomography finding was interpreted as liver hemangioma. However, based on liver biopsy, the diagnosis had to be changed to primary systemic amyloidosis, and the patient was referred to our hematooncology department. Due to a considerably advanced disease, the patient was eligible only for palliative chemotherapy with cyclophosphamide and dexamethasone, which could not deflect the course of rapidly progressing liver destruction. Conclusion: The cause behind ruptured and bleeding liver does not always need to be hemangioma but rather amyloidosis. In cases of advanced disease and in patients with contraindications for aggressive treatment, the outlook for complete hematological and organ treatment response is very limited. An early diagnosis is of utmost importance. Although liver biopsy brings the definite results, screening for monoclonal protein in serum or urine, leading to a search for AL amyloidosis, may be sufficient for diagnosis. The presence of some of the warning signs (B-symptoms such as fevers or subfebrile temperatures, fatigue, weight loss; and paraneoplastic laboratory findings such as elevated C-reactive protein and erythrocyte sedimentation rate) should raise suspicion of a lymphoproliferative disease.
ER -
SZTURZ, Petr, Jitka KYCLOVÁ, Mojmír MOULIS, Milan NAVRÁTIL, Zdeněk ADAM, Jiří VANÍČEK and Jiří MAYER. Extensive AL Amyloidosis Presenting with Recurrent Liver Hemorrhage and Hemoperitoneum: Case Report and Literature Review. \textit{Klinická onkologie}. Praha: Česká lékařská společnost J.E.Purkyně, 2013, vol.~26, No~1, p.~49– 52. ISSN~0862-495X.
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