Vnitr Lek 2019, 65(11):715-720 | DOI: 10.36290/vnl.2019.124

Pancytopenia, panhypopituitarism and liver cirrhosis: analysis of a difficult clinical case

Pavel Polák^1,2,*, Jan Kamelander¹, Jana Michalcová^1,2, Jiřina Zavřelová^1,3, Libor Červinek⁴, Tomáš Rohan⁵, Zuzana Pazdičová⁵, Václav Odstrčilík⁶, Miroslav Penka^1,2,3

¹ Oddělení klinické hematologie FN Brno, pracoviště Bohunice

² LF MU, Brno

³ Katedra laboratorních metod LF MU, Brno

⁴ Interní hematologická a onkologická klinika LF MU a FN Brno, pracoviště Bohunice

⁵ Klinika radiologie a nukleární medicíny LF MU a FN Brno, pracoviště Bohunice

⁶ Klinika ústní, čelistní a obličejové chirurgie LF MU a FN Brno, pracoviště Bohunice

Panhypopituitarism following craniopharyngioma resection has systemic impact with potential influence on physiological 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.

Keywords: coagulopathy; liver cirrhosis; pancytopenia; panhypopituitarism

Received: May 22, 2019; Accepted: September 9, 2019; Published: November 1, 2019  Show citation