Lymphomatoid Papulosis in a Patient with Waldenström's Macroglobulinemia

Abstract

Lymphomatoid papulosis (LyP) is a rare skin disease with a benign course but a malignant histologic appearance based on atypical lymphocytes. Waldenström's macroglobulinemia (WM) is a diffuse infiltration of the bone marrow by cells that synthesize IgM immunoglobulins. A 46-year-old female presented with a two year history of weakness, fatigue, anemia, and recurring multiple erythematous papules and nodules in the skin. The skin biopsy showed pleomorphic lymphoid cells with atypical mitoses permeated by a diffuse dermal infiltrate of normal appearing lymphocytes. The neoplastic cells were positive for CD30, CD3, and CD5 but negative for CD20 and EMA. The bone marrow was hypercellular due to a diffuse infiltration by lymphocytes, plasma cells, and plasmacytoid lymphocytes. LyP is a lymphoproliferative disorder of CD30-positive T cells that may be associated with other lymphoid malignancies, particularly Hodgkin's disease, mycosis fungoides, and anaplastic T cell lymphomas. To our knowledge, this is the first report of a LyP appearing in a patient with WM.

Intensely Pruritic Papules and Plaques in Waldenstrom's Macroglobulinemia

“…Patients with WM typically exhibit bone marrow with over 10% infiltration by clonal lymphoplasmacytic cells and a monoclonal IgM gammopathy in the blood. Various dermatologic conditions have been reported in association with WM and other monoclonal gammopathies such as multiple myeloma 1 5 . Dermatologic conditions with WM are rare 6 , appearing in only about 5% of cases.…”

“…It is characterized by the excess production of immunoglobulin M (IgM) monoclonal (M) protein, which gives the name “macroglobulinemia.” Common presenting symptoms include pallor, oronasal bleeding, weakness, fatigue, weight loss, fever, and night sweats. Various systemic and neurologic symptoms, such as impaired vision, headache, stroke, and dementia, may also be seen due to hyperviscosity syndrome 1 . Many dermatologic problems have been reported in association with M proteins 2 , specifically in WM and other monoclonal gammopathies.…”

Klinische Verlaufsformen und Therapie der lymphomatoiden Papulose

“…In einem weiteren Fall bestand gleichzeitig ein M. Waldenström. Das Zusammentreffen dieser beiden Lymphomerkrankungen wurde unseres Wissens bisher nur 1-mal in der englischsprachigen Literatur beschrieben[29]. In Übereinstimmung mit der Literatur konnte in unserem Patientengut diese nicht zu unterschätzende Assoziation der lymphomatoiden Papulose mit anderen Lymphomen bestätigt werden.…”

Aplicabilidade da classificação WHO 2008 para os linfomas de células T não-micose fungóide/síndrome de Sézary com expressão primária cutânea

“…A duração da doença pode variar de alguns meses a mais de 40 anos (Willemze et al, 1982). Em até 20% dos pacientes, pode ser precedida por, estar associada com ou ser seguida por outro tipo de neoplasia linfóide, consoante supramencionado (Bekkenk et al, 2000;Ribeiro-Silva et al, 2005;Kadin, 2006).…”