Paroxysmal Nocturnal Hemoglobinuria and Primary Myelofibrosis as an Extremely Rare Combination of Clonal Hematological Diseases: A Literature Review and Two Clinical Case Reports

Abstract

The combinations of paroxysmal nocturnal hemoglobinuria (PNH) and chronic myeloproliferative neoplasms (CMPNs) are extremely rare. All of them refer to clonal hematological diseases and are characterized by high thrombosis risk, which most commonly causes death. This paper provides literature data on 38 combined cases of PNH and Ph-negative/Ph-positive CMPNs mainly in the “case report” format, taken from 22 sources published in 1970–2022. Additionally, the paper reports personal experience with 2 combined cases of PNH and primary myelofibrosis (PMF/PNH) from the archive of the Moscow Municipal Center for Hematology (SP Botkin City Clinical Hospital).