A 30‐year‐old African American female diagnosed with biopsy‐confirmed hepatic sarcoidosis 10 years prior was admitted for asymptomatic pancytopenia and worsening transaminitis on routine outpatient testing. She had no other past medical history, and was remotely on prednisone but not currently adherent on any immunosuppressive medications. She reported no recent travel or toxic habits. Family history included an aunt and grandmother with sarcoidosis, and a cousin with systemic lupus. Except for splenomegaly, vitals and examination were largely unremarkable. Laboratory evaluation confirmed pancytopenia and elevated liver enzymes (WBC 0.91K/µL, absolute neutrophil count 0.49K/µL, hemoglobin 7.6g/dL, platelets 45K/µL, total bilirubin 1.6mg/dL, alkaline phosphatase 311u/L, AST/ALT 105/62u/L). Chest radiograph and CT showed no lung pathology. Abdominal ultrasound confirmed splenomegaly and revealed chronic portal vein thrombosis with splenic hilar/gastrohepatic varices, consistent with portal hypertension. Tests for additional causes of liver disease, including hepatitis, hemochromatosis, and Wilson’s disease, were negative. Serologic workup for other causes of her pancytopenia included HIV; hemoglobin electrophoresis; vitamin B12 and folate; quantiferon gold; and anti‐nuclear, anti‐double stranded DNA, parvovirus, CMV, EBV, and ribonucleoprotein antibodies, all of which were unremarkable. Bone marrow biopsy revealed normocellularity with trilinear hematopoiesis. Despite her laboratory abnormalities, the patient had no acute complications during her hospitalization. At discharge, she was referred for evaluation for splenectomy.
Sarcoidosis is a systemic granulomatous disease of unknown etiology that commonly affects the lungs, although virtually any organ can be involved. Portal hypertension as a complication of hepatic sarcoidosis has been reported in less than 1% of patients; while accompanying pancytopenia with splenomegaly is even more scarcely reported. We postulate that granulomatous involvement of the patient’s liver and spleen by underlying sarcoidosis resulted in development of portal vein thrombosis and subsequent hypertension, as well as pancytopenia. Evidence has shown that splenectomy reduces portal pressure (reducing the risk of variceal bleeding) and completely corrects the associated pancytopenia resulting from splenic sequestration.
Hospitalists face a compelling diagnostic challenge when admitting patients with pancytopenia, especially since the complications of pancytopenia are potentially life‐threatening if left untreated. This case highlights the approach to pancytopenia and the importance of considering extramedullary causes, particularly in the setting of systemic inflammatory disease.
To cite this abstract:Braunstein M, Lee M, Kumar D. Thinking Outside the Marrow: An Unusual Case of Pancytopenia in a Woman with Extrapulmonary Sarcoidosis. Abstract published at Hospital Medicine 2014, March 24-27, Las Vegas, Nev. Abstract 356. Journal of Hospital Medicine. 2014; 9 (suppl 2). https://www.shmabstracts.com/abstract/thinking-outside-the-marrow-an-unusual-case-of-pancytopenia-in-a-woman-with-extrapulmonary-sarcoidosis/. Accessed April 1, 2020.