Bone marrow histopathology in POEMS syndrome: A distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients

Linda N. Dao, Curtis A. Hanson, Angela Dispenzieri, William G. Morice, Paul J. Kurtin, James Hoyer

Research output: Contribution to journalArticle

55 Citations (Scopus)

Abstract

POEMS is an uncommon syndromic disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. There are few descriptions of the bone marrow pathology of POEMS; therefore, peripheral blood smears and bone marrow aspirates and biopsies from 87 patients (143 total, 67 pretreatment, 76 posttreatment cases) with POEMS were studied. Plasma cell clonality was analyzed by flow cytometry, immunohistochemistry, and/or in situ hybridization. Monotypic plasma cells were detected in 44 pretreatment cases (66%); the majority of plasma cells expressed λ light chain (91%). The monotypic plasma cells typically were present in a background of increased polytypic plasma cells. Lymphoid aggregates were found in 33 (49%) pretreatment cases and in most cases were rimmed by plasma cells (97%). Megakaryocyte hyperplasia (36 cases) and clusters (62 cases) were frequent; however, none of the 43 cases tested had the JAK2 V617F mutation. In summary, we have identified a novel constellation of features that should strongly suggest POEMS syndrome as part of the differential diagnosis. The constellation of λ-restricted monoclonal gammopathy, plasma cell rimming around lymphoid aggregates, and megakaryocytic hyperplasia in a bone marrow is highly suggestive of this diagnosis, especially in the context of a peripheral neuropathy.

Original languageEnglish (US)
Pages (from-to)6438-6444
Number of pages7
JournalBlood
Volume117
Issue number24
DOIs
StatePublished - Jun 16 2011

Fingerprint

POEMS Syndrome
Plasma Cells
Bone
Bone Marrow
Plasmas
Hyperplasia
Paraproteinemias
Megakaryocytes
Flow cytometry
Biopsy
Peripheral Nervous System Diseases
Pathology
In Situ Hybridization
Skin
Flow Cytometry
Differential Diagnosis
Blood
Immunohistochemistry
Light
Mutation

ASJC Scopus subject areas

  • Hematology
  • Biochemistry
  • Cell Biology
  • Immunology

Cite this

Bone marrow histopathology in POEMS syndrome : A distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients. / Dao, Linda N.; Hanson, Curtis A.; Dispenzieri, Angela; Morice, William G.; Kurtin, Paul J.; Hoyer, James.

In: Blood, Vol. 117, No. 24, 16.06.2011, p. 6438-6444.

Research output: Contribution to journalArticle

Dao, Linda N. ; Hanson, Curtis A. ; Dispenzieri, Angela ; Morice, William G. ; Kurtin, Paul J. ; Hoyer, James. / Bone marrow histopathology in POEMS syndrome : A distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients. In: Blood. 2011 ; Vol. 117, No. 24. pp. 6438-6444.
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abstract = "POEMS is an uncommon syndromic disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. There are few descriptions of the bone marrow pathology of POEMS; therefore, peripheral blood smears and bone marrow aspirates and biopsies from 87 patients (143 total, 67 pretreatment, 76 posttreatment cases) with POEMS were studied. Plasma cell clonality was analyzed by flow cytometry, immunohistochemistry, and/or in situ hybridization. Monotypic plasma cells were detected in 44 pretreatment cases (66{\%}); the majority of plasma cells expressed λ light chain (91{\%}). The monotypic plasma cells typically were present in a background of increased polytypic plasma cells. Lymphoid aggregates were found in 33 (49{\%}) pretreatment cases and in most cases were rimmed by plasma cells (97{\%}). Megakaryocyte hyperplasia (36 cases) and clusters (62 cases) were frequent; however, none of the 43 cases tested had the JAK2 V617F mutation. In summary, we have identified a novel constellation of features that should strongly suggest POEMS syndrome as part of the differential diagnosis. The constellation of λ-restricted monoclonal gammopathy, plasma cell rimming around lymphoid aggregates, and megakaryocytic hyperplasia in a bone marrow is highly suggestive of this diagnosis, especially in the context of a peripheral neuropathy.",
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