POEMS Syndrome and Small Lymphocytic Lymphoma Co-Existing in the Same Patient: A Case Report and Review of the Literature

Special Article - Hematology

Ann Hematol Oncol. 2015;2(1): 1019.

POEMS Syndrome and Small Lymphocytic Lymphoma Co-Existing in the Same Patient: A Case Report and Review of the Literature

Kasi Loknath Kumar A1,2*, Mathur SC3 and Kambhampati S1,2

1Department of Hematology and Oncology, Veterans Affairs Medical Center, USA

2Department of Internal Medicine, University of Kansas Medical Center, USA

3Department of Pathology, Veterans Affairs Medical Center, USA

*Corresponding author: Kasi Loknath Kumar A, Department of Internal Medicine, Division of Hematology and Oncology, University of Kansas Medical Center, Kansas City, 2330 Shawnee Mission Parkway, MS 5003, Suite 210, Westwood, KS, 66205, Kansas, USA

Received: November 25, 2014; Accepted: January 06, 2015; Published: January 08, 2015


The coexistence of B-cell Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma (CLL/SLL) and Plasma Cell Dyscrasias (PCD) has rarely been reported. The patient described herein presented with a clinical course resembling POEMS syndrome. The histopathological evaluation of the bone marrow biopsy established the presence of an osteosclerotic plasmacytoma despite the absence of monoclonal protein in the peripheral blood. Cytochemical analysis of the plasmacytoma demonstrated monotypic expression of lambda (λ) light chains, a typical finding associated with POEMS syndrome. A subsequent lymph node biopsy performed to rule out Castleman's disease led to an incidental finding of B-CLL/SLL predominantly involving the B-zone of the lymph node. The B-CLL population expressed CD19, CD20, CD23, CD5, HLA-DR, and kappa (κ) surface light chains. To the best of our knowledge, a simultaneous manifestation of CLL/SLL and POEMS has not been previously reported in the literature. The expression of a different immunoglobulin (Ig) light chain on the plasmacytoma (λ) and CLL (κ) suggested a biclonal B-cell origin. In our patient, a definite clonal relationship between the two neoplasms by Ig heavy chain gene rearrangements could not be established because of the nonsecretory myeloma and absence of CLL lymphocytosis in the peripheral blood.

Keywords: POEMS; CLL/SLL; Clonality; Gene rearrangement


VEGF: Vascular Endothelial Growth Factor; TNF-a: Tumor Necrosis Factor-Alpha; IL-1β: Interleukin 1-Beta; IL-6: Interleukin-6; PSA: Prostate Specific Antigen; HIV: human immunodeficiency virus.


POEMS is an acronym coined for a rare multisystem plasma cell neoplasm characterized by polyneuropathy, organomegaly, endocrinopathy, Monoclonal paraprotein (M protein), and skin changes [1-4]. Although there are several other associated features that are not included in the acronym, the most consistent clinical feature is that of a chronically progressive peripheral polyneuropathy [1-4]. Another defining element of this entity is the presence of a monoclonal gammopathy, which is usually an IgA-λ or IgG-λ [1- 4]. While the clinical spectrum of Plasma Cell Dyscrasia (PCD) in POEMS is variable, the most common subtype reported in all large series is the Osteosclerotic Myeloma (OSM) [1-4]. The molecular basis for such diverse manifestations seen in POEMS has not yet been defined. However, it is clear that the neoplastic clone of this plasma proliferative disorder expresses a pattern of somatic mutation in the Variable (V)-region genes that have the signature of a neoplasm derived from the post-germinal B-cells [5]. These proliferating monoclonal plasma cells then produce an antibody-mediated attack against neural antigens and secrete inflammatory cytokines, such as VEGF, TNF-a, IL-1β and IL-6 that are directly or indirectly responsible for the pathogenesis of this syndrome [3,4,6-9].

Unlike PCD, the clonal evolution of the most common B-cell derived malignancy Chronic Lymphocytic Leukemia (CLL)/Small Lymphocytic Leukemia (SLL) has been shown to be remarkably complex. Many groups have published work within the past decade suggesting that CLL is not simply a homogenous disease of slowlyproliferating and self-renewing B cells [10-12]. Rather, CLL, these days, is sub classified on the basis of the mutation status of V genes and the expressions of CD 38 and ZAP-70 [10-12]. Due to differences in the cellular evolution of CLL/SLL and PCD, these cancers rarely coexist in the same individual [10]. In those rare cases where CLL and PCD are coexistent, questions remain as to whether or not these cancers are clonally related. Presently, there are no definite conclusions. In this report we present a patient with POEMS syndrome in which B-CLL/SLL was found to be coexisting. Such a finding of concurrent POEMS and CLL has not been previously reported in literature.

Case Report

A 77-year-old Caucasian male was referred to the Hematology department at the Veteran Affairs (VA) Medical Center in Kansas City, Missouri for the evaluation of lymphadenopathy and osteosclerotic spinal lesions. His past medical history was extensive and most significant for the resection of a pituitary macro adenoma that resulted in panhypopituitarism. In addition, the patient also had a ten year history of progressive Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), which the Electromyography study (EMG) confirmed as an axonal sensor motor polyneuropathy. At the timeof initial evaluation, physical examination showed right posterior cervical and left axillary lymphadenopathy, diminished breath sounds with percussion dullness at the lung bases, and bilateral lower extremity edema. Abdomen was soft and without organomegaly. The patient was wheelchair bound due to an areflexic, grade 4/5 lower extremity weakness. Computed Tomography (CT) scan showed bilateral pleural effusions as well as extensive sclerotic lesions in the thoracolumbar spine, ribs, and pelvis. The results of a technetium bone scan were normal but a metastatic skeletal survey showed diffuse osteosclerosis in the skull and vertebral bodies (Figure 1).