Incidence and Characteristics of Lymphoid Malignancies in Untreated Myelodysplastic Syndromes

Abstract

We have analyzed 1,198 patients with untreated myelodysplastic syndromes (MDS) with two main objectives: (1) to determine the prevalence of lymphoid malignancies (LM) in MDS patients; and (2) to ascertain whether there is some relationship between the MDS subtype and the LM type. In fourteen of 1,198 primary MDS patients (1%) (4 with refractory anemia, 3 with refractory anemia with ring sideroblasts, 2 with refractory anemia with excess of blasts and 5 with chronic myelomonocytic leukemia) a LM was detected. In all cases, the LM was of the B-cell type: 6 cases of chronic lymphocytic leukemia, 5 cases of lymphoplasmacytoid lymphoma, and 3 cases of multiple myeloma. B-cell malignancy did not prevail in any MDS subtype and no correlation was observed between the different varieties of both diseases. In conclusion, in this large series, 1% of the untreated patients with MDS had B-cell malignancy, an association that in most cases is likely to be merely coincidental.

Simultaneous and Sequential Concurrent Myeloproliferative and Lymphoproliferative Neoplasms

“…However, in some MPN patients, apparently normal lymphocytes can be positive for JAK2 V617F or Ph + without generating an overt malignant lymphoma [14,93,94,95]. Similar to MPN, lymphocytes in MDS can be monoclonal [96], but the frequency of primary MDS with concurrent lymphoma is low (n = 14/1,198, 1%) [12]. Remarkably, in two small Ph – MPN series, 31% of patients (n = 5/16) [14,94] had JAK2 V617F -positive B cells (rarely JAK2 V617F T cells and no lymphomas), indicating that lymphoid sub-differentiation of an early common stem cell progenitor is not uncommon [14,94,97].…”

“…Myeloid-lymphoid phenotype switch is known during blastic transformation of Ph + CML but is extremely rare in other myeloid neoplasms or lymphomas [9,10]. Concurrent manifestation of two chronic-phase myeloid and lymphoid neoplasms in one patient is rare and occurs in approximately 1% of patients [11,12]. Several case reports and a few case series have addressed this issue [13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91].…”

T-cell Non-Hodgkin lymphoma associated with myelodysplasia: A case report in a child

“…A high incidence of MDS was reported in relation with solid tumors, such as lung, colon, prostate and liver cancers (11). The same relationship was described between MDS and lymphoid neoplasms, such as acute lymphoblastic leukemia, chronic lymphocytic leukemia, and NHL (12)(13)(14)(15). In 1996, a group of Spanish investigators studied the association of lymphoid malignancy in patients with primary MDS and found an association rate of only 1% and concluded that this association could be a coincidence (14).…”

Hodgkin's disease coexisting with myelodysplastic syndrome prior to therapy: a case report and a review of the association of Hodgkin's disease with stem cell disorders

“…Solid tumors such as carcinomas of lung, colon, prostate, and kidney have been reported at a higher incidence in association with MDS [21]. A similar association between MDS and lymphoid neoplasms such as acute lymphoblastic leukemia (ALL), non-Hodgkin's lymphoma (NHL), plasma cell neoplasms and chronic lymphocytic leukemia (CLL) has been reported [1,7,9,12]. On the other hand, Hodgkin's disease (HD) has been associated with a higher incidence of developing secondary MDS and acute leukemia following chemotherapy and radiation therapy [14].…”