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Chronic myelomonocytic leukemia (CMML) is a rare, neoplastic hematopoietic stem cell disorder characterized by persistent monocytosis in the peripheral blood together with both myelodysplastic and myeloproliferative features in the bone marrow. Necrotising sarcoid granulomatosis (NSG) is an uncommon systemic inflammatory disorder characterized by sarcoid-like granulomas, vasculitis, and varying degrees of necrosis. A 69-year-old-female presenting in the emergency department with complaints compatible with pneumonia who also had a two year history of frequently recurring respiratory tract symptoms and concomitant findings of elevated erythrocyte sedimentation rate is presented herein. The patient who was initially evaluated for causes of reactive monocytosis was eventually diagnosed as NSG with findings on lymph node biopsy. While a favourable clinical response was observed to the glucocorticoid therapy initiated for NSG, no improvements were detected in her hematological tests. The patient’s bone marrow examination and genetic analysis were performed and it was documented that the co-occurrence of CMML was underlying the patient’s unremitting monocytosis and other accompanying hematological test abnormalities. On this patient’s occasion, the differential diagnosis and the association of the two unusual and distinct causes of monocytosis are reviewed in light of the current literature.

Association of the Rares: the Occurrence of Chronic Myelomonocytic Leukemia During the Course of Necrotising Sarcoid Granulomatosis