The majority of VEXAS individuals had recurrent fevers, arthralgias/arthritis, neutrophilic dermatosis and cutaneous vasculitis, pulmonary involvement (Supplementary Table 1), macrocytic anemia (Fig. 1C), leukopenia, mainly lymphocytopenia and monocytopenia, and increased C-reactive protein and lactate dehydrogenase levels (Fig. 1D), and bone marrow vacuolization restricted to myeloid and erythroid precursor cells (Fig. 1E). Here, CRP is linked to Decreased total monocyte count.