Simultaneous and Sequential Concurrent Myeloproliferative and Lymphoproliferative Neoplasms

Abstract

Concurrent manifestation of two chronic-stage myeloid and lymphoid/plasmacytoid neoplasms in one patient is rare and occurs in ≤1% of patients. There has been no systematic analysis of which combinations are frequent/infrequent and whether two concurrent diseases in one patient are clonally related or represent independent diseases. We therefore characterised a series of cases from our own archive (n = 65) and collected a large number of previously reported cases of patients in whom myeloid and lymphoid/plasmacytoid neoplasms co-occurred (n = 185). The most frequent combination was Philadelphia chromosome-negative myeloproliferative neoplasm with concurrent B cell chronic lymphocytic leukaemia, accounting for approximately 50% of double-disease patients. We compared the quantity of unsorted bone marrow cell-derived JAK2^V617F and KIT^D816V alleles with the quantity of the lymphoid/plasmacytoid compartment and analysed a subfraction of cases with fluorescence in situ hybridisation. Although a common aberrant progenitor has been reported in some cases in the literature, we found evidence of two independent chronic-stage myeloid and lymphoid/plasmacytoid neoplasms.

Quantification of JAK2V617F mutation by next‐generation sequencing technology

“…Earlier clinical studies have likewise demonstrated a beneficial effect in early stage B-CLL using nonpegylated interferon alpha 2a [3]. The diagnosis of concomitant CLL/MPN is a rare event and in a recent study analysis of prognostic markers for CLL demonstrated that in patients with a coexistent MPN, lymphoproliferative disorders follow a clinically indolent course [4,5].Our patient has achieved a substantial hematologic, molecular and splenic response in his PV after 22 months on low dose Pegasys. Moreover, he has achieved a favorable reduction in the B-cell CLL clonal population and a change in Zap-70 status from indeterminate to negative.…”

“…Earlier clinical studies have likewise demonstrated a beneficial effect in early stage B-CLL using nonpegylated interferon alpha 2a [3]. The diagnosis of concomitant CLL/MPN is a rare event and in a recent study analysis of prognostic markers for CLL demonstrated that in patients with a coexistent MPN, lymphoproliferative disorders follow a clinically indolent course [4,5].…”

Mutations and chromosomal rearrangements ofJAK2: not only a myeloid issue

“…A recent study conducted on a total of 250 cases reported that the combination of a MPN with a B-CLL accounts for a 50% of the double-disease patients analyzed [72]. Moreover, in these cases the evaluation of JAK2V617F in different lineages is of increasing interest as it could help to understand if the myeloproliferative and lymphoproliferative disorders are both derived from the same pluripotent stem cell or just coincide.…”

A rare case of primary cutaneous follicle centre lymphoma presenting as a giant tumour of the scalp and combined with JAK2V617F positive essential thrombocythaemia

“…Only a few cases of concomitantly occurring MPN and LPN have been reported [3-8]. Laurenti et al published a multicenter retrospective study on the association of chronic lymphocytic leukemia (CLL) and concomitant MPN [9] but at present, no systematic analysis for MPN-LPN disease association is available and underlying pathogenic mechanisms remain unclear.…”