A Rare Case of Relapsed Sarcoidosis Presenting As Severe Thrombocytopenia Associated With Intracerebral Hemorrhage Due to Bone Marrow Involvement

Abstract

Sarcoidosis is a systemic granulomatous disease characterized by the hyperactivation of CD4 T cells, CD8 T cells, and macrophages. Clinical presentations of sarcoidosis are highly variable. Sarcoidosis is unknown in its etiology, but it suggests it may result from exposure to specific environmental agents in genetically susceptible people. Sarcoidosis commonly involves the lungs and lymphoid system. Bone marrow involvement in sarcoidosis is uncommon. Sarcoidosis rarely results in intracerebral hemorrhage due to severe thrombocytopenia secondary to bone marrow involvement. We present the case of a 72-year-old woman who has been in remission from sarcoidosis for 15 years and developed intracerebral hemorrhage secondary to severe thrombocytopenia due to sarcoidosis recurrence in the bone marrow. The patient presented to the emergency department with a generalized, non-blanching petechiae rash and nose and gum bleeding. Her labs showed a platelet count of less than 10.000/mcL, and computed tomography (CT) showed intracerebral hemorrhage. A bone marrow biopsy revealed a small, non-caseating granuloma indicative of a sarcoidosis relapse in the bone marrow.

Keywords: bone marrow involvement; bone marrow sarcoidosis; granulomatous disease; intracerebral hemorrhage; petechiae rash; relapsed sarcoidosis; sarcoidosis; severe thrombocytopenia.

Figure 1. A chest x-ray showed bilateral upper lung predominant fibrotic changes (green arrows).

Figure 2. A computerized tomography (CT) of the head showed a 4 mm intracranial hemorrhage in the right frontal lobe.

Figure 3. A chest CT without contrast showed extensive scarring and fibrosis within the apical prominence (green arrows pointed to scarring and fibrosis; A, B: axial views at different sections of the lungs).

Figure 4. CT chest without contrast (coronal view) showed extensive scarring and fibrosis within apical prominence (green arrows pointing to scarring/fibrosis).

Figure 5. A section of the bone marrow biopsy (H and E x4) showed a small focus of non-necrotizing granuloma (black arrow).

H&E: hematoxylin and eosin stain

Figure 6. A section of the bone marrow biopsy showed the granuloma at higher magnification with epithelioid histiocytes that have fused to form giant cells surrounded by mononuclear lymphocytes (H and E x20).