Fig. 1

Schematic diagram summarizing the evolution of MonoMAC syndrome. Evolutional timeline of history and results of MonoMAC syndrome patient. Each ball represents a year; he initially presented pancytopenia, monocytopenia and myelodysplastic syndrome (MDS) in 1996 with 30 years-old; at 43 years-old systemic Mycobacterium kansaii infection developed and persisted until 2015, when refractory anemia with excess of blasts (RAEB2) and MonoMAC syndrome were diagnosed. Nitro blue tetrazolium (NBT). In 2016 he was submitted to hematopoietic stem cell transplant (HSCT) and died with 50 years-old. Pictures represents (a) Thoracic Computed Tomography with pulmonary opacification; b Painless perimalleolar ulcers; c Knee arthritis; d Granulomatous splenic inflammation, necrosis and calcification (H&E stain); e Bone marrow dysplasia with small and hypolobulated megakaryocytes and myelofibrosis (H&E stain of core biopsy) f Nonspecific skin spores and septate hyphae (Groccot strain)