Case Report

Plasmablastic Lymphoma in an Immunocompetent Patient with MDS/MPN with Ring Sideroblasts and Thrombocytosis—A Case Report

Table 1

Diagnostic criteria for MDS/MPN with RS-T according to WHO 2016 classification [3].

Diagnostic criteria

(i) Anemia associated with erythroid lineage dysplasia with or without multilineage dysplasia ≥15% sideroblasts, <1% in PB, and <5% blasts in BM
(ii) Persistent thrombocytosis with platelet count ≥450 × 109/L
(iii) Presence of SF3B1 mutation or in the absence of SF3B1 mutation, no history of recent cytotoxic or growth factor therapy that could explain the myelodysplastic/myeloproliferative features
(iv) No BCR-ABL1 fusion gene, no rearrangement of PDGFRA, PDGFRB, or FGFR1; PCM1-JAK2, no t(3;3) (q21; q26), inv(3) (q12;q26) or del(5q)
(v) No preceding history of MPN, MDS (except MDS-RS), or other type MDS/MPN

At least 15% ring sideroblasts required even if SF3B1 mutation is detected. A diagnosis of MDS/MPN-RS-T is strongly supported by the presence of SF3B1 mutation together with a mutation in JAK2 V617F, CALR, or MPL genes. In a case which otherwise fulfills the diagnostic criteria for MDS with isolated del(5q-), no or minimal absolute basophilia, basophils usually 2% of leukocytes.