Pancytopenia, panhypopituitarism and liver cirrhosis: analysis of a difficult clinical case
| Autor: | Pavel Polák, Jana Michalcová, Jiřina Zavřelová, Jan Kamelander, Václav Odstrčilík, Miroslav Penka, Tomáš Rohan, Libor Červinek, Zuzana Pazdičová |
|---|---|
| Rok vydání: | 2019 |
| Předmět: |
medicine.medical_specialty
Cirrhosis biology business.industry 030209 endocrinology & metabolism medicine.disease Chronic liver disease Pancytopenia Gastroenterology Protein S 03 medical and health sciences 0302 clinical medicine 030220 oncology & carcinogenesis Internal medicine Internal Medicine medicine Paroxysmal nocturnal hemoglobinuria biology.protein Portal hypertension Cardiology and Cardiovascular Medicine Viral hepatitis business Hemochromatosis |
| Zdroj: | Vnitřní lékařství. 65:715-720 |
| ISSN: | 1801-7592 0042-773X |
| DOI: | 10.36290/vnl.2019.124 |
| Popis: | Panhypopituitarism following craniopharyngioma resection has systemic impact with potential influence on physio-logical hematopoiesis. There is a growing body of evidence of liver fibrosis/cirrhosis risk development due to altered metabolism and lipid accumulation. The authors present a case report of a woman with a history of craniopharyngioma resection followed by aggravating pancytopenia with suspected indolent lymphoproliferative disorder and possible acquired bone marrow aplasia syndrome due to paroxysmal nocturnal hemoglobinuria. A complex hemostasis disorder with deficiency of multiple coagulation factors (FXII, FXI, FX, FIX, FVII, FX, FV, FXIII, antitrombin, protein C, protein S) was accidentally detected. Despite normal sonographic liver imaging, all possible causes of chronic liver disease were systematically excluded (viral hepatitis, hemochromatosis, Wilson´s disease, α-1-antitrypsin deficiency); anti-LKM-1 and anti-ENA antibodies were detected. Finally, the magnetic resonance imaging confirmed image of liver cirrhosis - with signs of portal hypertension. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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