Abstract
The coexistence of a myeloid and a lymphoid neoplasm in the same patient is a rare finding. We retrospectively searched the records of the Hematology Division of the Second Department of Internal Medicine and Research Institute at Attikon University General Hospital of Athens from 2003 to 2018. Nine cases have been identified in a total of 244 BCR-/ABL1- negative MPN and 25 MDS/MPN patients and 1062 LPD patients referred to our institution between 2003 and 2018. Each case is distinct in the diversity of myeloid and lymphoid entities, the chronological occurrence of the two neoplasms, and the patient clinical course. All of them exhibit myeloproliferative (6 JAK2 V617F-positive cases) and lymphoproliferative features, with 1 monoclonal B-cell lymphocytosis (MBL), 3 B-chronic lymphocytic leukemias (B-CLL), 3 B-non-Hodgkin lymphomas (B-NHL), 1 multiple myeloma (MM), and 1 light and heavy deposition disease (LHCDD), while in three cases myelodysplasia is also present. The challenges in identifying and dealing with these rare situations in everyday clinical practice are depicted in this article.
Highlights
Introduction e annual incidence ofBCR-/ABL1-negative myeloproliferative neoplasms (MPN), namely polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) in the western world, is estimated to be maximum 2.8, 2.3, and 1.5 cases per 100.000 population, respectively [1, 2]
In a study by Vannucchi et al, which included 820 BCR-/ABL1negative MPN patients in a period from 1980 to 2008, 11 concomitant lymphoproliferative disorders (LPD) cases were reported, with a 3.44-fold increase in the risk of developing LPD in the MPN group compared with the general population [4]
We have presented a case series of patients with diverse MPN with or without myelodysplastic features coexisting with various LPD
Summary
Patient 7 had been supported with RBC transfusions and erythropoiesis-stimulating agents for her MDS/MPN-RS-T She only received 2 cycles of obinutuzumab/chlorambucil due to a dramatic increase in her transfusion needs. It should be noted that the diagnosis of the MPN preceded that of LPD in all patients with the exception of Patient 4 Regarding this patient, a splenic MZL was diagnosed in another institution based on spleen pathology, and at this time point, bone marrow biopsy was reported to be normal and no further information was available in his records. Is patient, having a 4-year history of JAK2-positive post-ET MF, developed persistent diarrheas and acute renal failure Her serum protein electrophoresis and immunofixation were normal, and her bone marrow exhibited no plasmacytic infiltration.
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