Waldenstrom's macroglobulinemia transformed into immunoblastic lymphoma presenting with malignant ascites

Abstract

Letters and Correspondence 249gous to Richter's syndrome occurring with chronic lymphocytic leukemia [ 11. Transformation is signaled by diffuse lymphadenopathy, fevers, night sweats, and weight loss [2]. To our knowledge, we report the first case of a patient with malignant ascites as a presenting manifestation of WM that transformed into L.A 57-year-old male with a history of WM presented with 2 weeks of progressive abdominal distention, cervical adenopathy, fevers, night sweats, and weight loss. Ten years previously, he was diagnosed with WM. Immunocytochemistry of a marrow specimen demonstrated the majority of cells to be IgM kappa-positive. He received multiple agents, including cyclophosphamide, vincristine, prednisone, and chlorambucil. He had no history of ethanol use or liver disease.Physical examination revealed left-sided cervical, supraclavicular. and axillary adenopathy. The abdomen was distended with shifting dullness. Serum lactate dehydrogenase (LDH) was 1,s 17 uniM and plasma viscosity was 2.87 (nl 0.99-1.55). White blood cell count was 24,000 cells/mm' with 30% immunoblasts, suggesting transformation of WM into a more aggressive neoplasm. Flow cytometry of these cells was consistent with WM. Paracentesis revealed numerous cells similar in morphology to the immunoblasts in the peripheral blood (Fig. 1); the culture was sterile. Ultrasound of the liver was normal. Computerized tomography scan showed massive thoracic and abdominal lymphadenopathy. A cervical lymph node biopsy was consistent with 1L and stained positive for IgM kappa, supporting clonal transformation. The patient denied further intervention and died 13 days after admission.Transformation of WM into IL is a rare event, occurring in approximately 1.7% of cases of WM [3]. The mean survival once transformation occurs in 2 months [2]. hior therapy with alkylating agents may predispose to transformation. though this remains to be proven [3]. IL is characterized by the monomorphous proliferation of immunoblasts. which are large cells with plasmacytoid features [I]. Both WM and IL are clonal diseases, and the change in histologic appearance of the cells characteristic of WM into the larger cells of IL is believed to represent evolution of the same clone of cells. Furthermore, the appearance of identical heavy and light chains on both tumor cell types supports clonal evolution [4].Ascites due to IL is very rare. Peritoneal lymphomatosis is much less common than peritoneal carcinomatosis and carries a dismal prognosis [5]. Runyon and Hoefs [5] reported on three patients with malignant ascites as could be obtained by shortening the duration of OC administration. No interferences with hemostatic balance are documented.

Diffuse Large B-Cell Lymphoma Occurring in Patients With Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia

“…Similarly, patients with LPL/WM may develop DLBCL, as has been described in isolated case reports and small series. [2][3][4][5][6][7][8][9][10] However, a large series of these cases has not been reported. Furthermore, risk factors for developing DLBCL in patients with LPL/WM are poorly understood.…”

Multiple Brain Tumors of Diffuse Large B Cell Lymphoma in a Patient with Waldenstroem's Macroglobulinemia/Lymphoplasmacytic Lymphoma: PCR and DNA Sequence Analysis Show Evidence of Differences in Clonality of the Two B Cell Malignancies

“…In 1936 about DLBL co-occurring with WM/LPL are that DLBL is a therapy-related (alkylating agent) secondary malignancy, and that there is a spontaneous association of DLBL with WM/LPL in a susceptible host (6,8). …”

“…WM/LPL is rarely complicated by other types of B cell malignancies and its prognosis is poor (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12).…”

Lymphoplasmacytic lymphoma presenting as malignant ascites

“…While we found several previously reported cases in the literature with the primary presenting symptom of lymphoma as ascites, only two cases were diagnosed as LPL/WM [4 -6]. One additional related case of a patient with a history of WM where immunoblastic transformation was heralded by new onset ascites was also noted [7].…”