Castleman disease variant of POEMS syndrome without M protein: a case report.

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) syndrome is a paraneoplastic syndrome associated with an underlying plasma cell neoplasm. According to the current diagnostic criteria for POEMS syndrome, the presence of characteristic polyneuropathy and clonal plasma cell disorder are required for diagnosis. We report a case of a Castleman disease variant of POEMS syndrome without monoclonal protein (M protein) expression, which presented with polyneuropathy, organomegaly, endocrinopathy, skin lesions, and sclerotic bone lesions.

First-line therapy with daratumumab, lenalidomide and dexamethasone for patient with POEMS syndrome: A case report.

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes syndrome) is a rare condition due to an underlying plasma cell neoplasm whose clinical presentation can be various so it could lead to delayed diagnosis and treatment. The pathogenesis of the syndrome is not well understood, and its therapy is adapted from other plasma cell disorders with the aim of alleviating symptoms, decreasing end-organ damage, improving quality of life and prolonging overall survival. We report a case of a 71 years-old woman who has been treated with continuous DRd (daratumumab, lenalidomide and dexamethasone) scheme.

Capillary leak phenotype as a major cause of death in patients with POEMS syndrome.

Cause of death (COD) in POEMS (polyneuropathy, organomegaly, endocrinopathies, monoclonal protein and skin changes) syndrome is not well described. We investigated COD in patients with POEMS syndrome treated at Mayo Clinic between 2000 and 2022. Of the 89 deaths, 49 patients had known COD and were the subject of this study.

Castleman disease-type histopathological patterns of lymph nodes in patients with plasma cell neoplasia and POEMS syndrome.

Plasma cell neoplasia and POEMS syndrome patients may present Castleman disease (CD)-type features in lymph nodes. Our aim was to better characterize the histopathological patterns found in plasma cell neoplasia associated CD and to improve the detection of clonal plasma cell populations in the lymph node biopsies of these patients. Lymph node and bone marrow samples from six cases with plasma cell neoplasia associated CD, including POEMS syndrome and multiple myeloma were analyzed.

Polyneuropathy With Motor Conduction Block in POEMS.

Introduction/aims: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell dyscrasia. Polyneuropathy in POEMS syndrome may present as a subacute or chronic symmetric sensorimotor polyneuropathy, with electrophysiological features suggesting demyelination. Motor conduction block (CB), which is mostly seen in chronic inflammatory demyelinating polyneuropathy (CIDP), is considered an atypical electrophysiological feature in POEMS syndrome.

Osteosclerosing multiple myeloma: A case highlighting critical value of image findings for the diagnosis.

Osteosclerosing myeloma is a specific form of multiple myeloma. We present a case of a 63-year-old woman who was found to have anemia during her medical checkup. Her blood tests suggested multiple myeloma, but she had diffuse osteosclerotic changes mainly in the axial skeleton, such as osteoblastic bone metastasis.

[POEMS syndrome. Report of three cases and literature review].

Background: POEMS syndrome is a debilitating paraneoplastic disorder associated with plasma cell neoplasia. Clinically, it is characterized by predominantly demyelinating peripheral neuropathy that often starts in the pelvic limbs, organomegaly, endocrinopathy, skin changes, and elevated serum M protein. Diagnosis requires meeting a set of clinical criteria in which polyradiculoneuropathy and plasma cell disorder are the two mandatory criteria.

Disease Characteristics and Outcomes of 493 Young Myeloma Patients Treated With Modern Therapies: A Canadian Myeloma Research Group Database Study.

Background: Young patients ≤ 50 years old with multiple myeloma (MM) account for about 10% of cases and are underrepresented in the literature.

Methods: We explored disease characteristics, treatments, and outcomes following modern therapies of young MM patients using the Canadian Myeloma Research Group (CMRG) database. We included 493 patients ≤ 50 years old diagnosed with MM or plasma cell leukemia without concurrent amyloidosis or POEMS syndrome from January 1, 2010, to July 1, 2022.

[POEMS Syndrome: Report of Two Cases].

POEMS syndrome is a multisystem disorder caused by a neoplasm of plasma cells. The acronym "POEMS" refers to some of its characteristics: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. It's a rare condition, and available information has been presented as case series.

Top 10 Clinical Pearls in Paraproteinemic Neuropathies.

Paraproteinemic neuropathies represent an important subset of peripheral neuropathies. Once identified, further evaluation into the paraproteinemic subtype, clinical exam pattern, and electrodiagnostic phenotype helps clarify if the paraproteinemia is coincidental or causal of the neuropathy, as . Of all paraproteinemias, immunoglobulin M (IgM)-associated peripheral neuropathy, or IgM neuropathy, is of particular importance as half of IgM neuropathies also harbor anti-myelin-associated glycoprotein antibodies, which produce a characteristic demyelinating pattern on nerve conduction testing.

Castleman Disease: A Rare Lymphoproliferative Disorder With Diverse Clinical Presentation, Diagnosis, and Treatment Approach.

Castleman disease (CD) includes rare and intricate lymphoproliferative disorders characterized by the abnormal growth of lymph nodes and immune system disturbances. It primarily presents in two forms: unicentric Castleman disease (UCD), which affects a single lymph node area, and multicentric Castleman disease (MCD), which involves multiple lymph nodes and systemic manifestations. The disease's underlying mechanisms are often linked to immune system irregularities, especially involving interleukin-6 (IL-6).

Acute leukoencephalopathy associated with daratumumab treatment in POEMS syndrome: a case report.

Objectives: Daratumumab, a monoclonal antibody against CD38, is increasingly used in the treatment of multiple myeloma, other hematological malignancies and autoimmune diseases. Little is known about its CNS toxicity. We present a case of a patient with POEMS syndrome (syndrome of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) who developed an acute leukoencephalopathy shortly after initiation of therapy with daratumumab.