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Our patient had almost all the features of POEMS are also three histological forms of CD: (1) hyaline
syndrome, including multiple peripheral neuropathy, vascular form, (2) plasma cell form, and (3) mixed.
splenomegaly, and multiple enlarged peripheral lymph Multicentric Castleman disease (MCD) is generally
nodes, diabetes, hypothyroidism, monoclonal IgG-λ the plasma cell type, but the hyaline vascular type
paraprotein, skin hyperpigmentation, and hypertrichosis. has been described in some patients. [19] Localized
Meanwhile, our patient also presented with systemic Castleman disease usually presents as masses in young
signs such as fever, abdominal distention, pitting adults (20–30 years of age). Systemic symptoms are rare
edema of the lower extremities, hydropericardium, in localized Castleman disease patients. In contrast,
hydrothorax, thrombocytosis, hypoalbuminemia, and MCD develops in old patients (40–50 years of age). The
increased fibrinogen levels. In brief, this patient is involvement of multiple lymph nodes and organs is
consistent with the standard diagnostic standard of frequent. [20] Our older patient presented with systemic
POEMS syndrome. symptoms and multiple enlarged lymph nodes. The
histological findings in this case are consistent with the
Castleman disease (CD, or angiofollicular lymph hyaline vascular form of Castleman disease.
node hyperplasia) is also a rare lymphoproliferative
disorder. [15] Castleman disease was first described by Castleman disease and POEMS syndrome are closely
Castleman et al. [16] in 1956. According to previous related. An association with MCD was reported in about
studies, the pathological feature of Castleman disease 50% of patients with POEMS. [6,19,20] Of 113 patients
is reactive proliferation in the lymphoid tissues. [17] The with MCD, 32% presented with criteria sufficient for
clinical features of Castleman disease are classified into a diagnosis of POEMS syndrome. [21] Here, our patient
two categories: localized and multicentric. [18] There presented with POEMS syndrome in association with
Castleman disease.
Table 1: Criteria for the diagnosis of POEMS syndrome [11]
Criteria/other symptoms and signs Affected, %* The pathogeny of POEMS syndrome remains unclear.
Mandatory major criteria (both required) It is assumed that hepatitis B antigen may play a role in
Polyradiculoneuropathy (typically demyelinating) 100 the etiology of this lymphatic disorder. [22] Our patient
Monoclonal plasma cell disorder (almost always λ) 100 †
Other major criteria (1 required) was positive for HBV. A previous study confirmed that
Castleman disease ‡ 11–25 increased levels of tumor necrosis factor alpha (TNF-α),
Sclerotic bone lesions 27–97 interleukin-6 (IL-6), and VEGF in patients of POEMS
VEGF elevation §
Minor criteria (1 required) syndrome are correlated with disease activity. [23]
Organomegaly (splenomegaly, hepatomegaly, or 45–85 However, we did not assess TNF-α, IL-6, or VEGF.
lymphadenopathy) Therapy for POEMS syndrome should include radiation,
Extravascular volume overload (edema, pleural 29–87
effusion, or ascites) chemotherapy, peripheral blood stem cell transplant,
Endocrinopathy (adrenal, thyroid, pituitary**, 67–84 targeting therapy, intravenous gamma-globulin therapy,
gonadal, parathyroid, pancreatic**) plasmapheresis, corticosteroids, etc. [11] The clinical
Skin changes (hyperpigmentation, hypertrichosis, 68–89
glomeruloid hemangiomata, plethora, acrocyanosis, course of POEMS syndrome is also chronic. A previous
flushing, white nails) study revealed that the median survival time of patients
Papilledema 29–64 with POEMS syndrome is 165 months. Another study
[6]
Thrombocytosis/polycythemia*** 54–88
Other symptoms and signs reported that the prognosis of MCD patients was poor,
Clubbing, weight loss, hyperhidrosis, pulmonary demonstrating a median survival time of 30 months. [20]
hypertension/restrictive lung disease, thrombotic
diatheses, diarrhea, low vitamin B values
12
The diagnosis of POEMS syndrome is confirmed when both of the mandatory The diagnosis of POEMS syndrome is often delayed
major criteria, 1 of the 3 other major criteria and 1 of the 6 minor criteria are because the syndrome is rare and can be mistaken for
present. *Summary of frequencies of POEMS syndrome features based on largest other neurological disorders. Thus, we hope our patient
retrospective series, [2,3,6‑9] † Takasuki and Nakanishi series are included, even though
only 75% of patients had a documented plasma cell disorder. Because these are with the Castleman disease variant of POEMS syndrome
among the earliest series describing the syndrome, they are included, There is a will prompt the universal recognition of this disease.
‡
Castleman disease variant of POEMS syndrome that occurs without evidence of a
clonal plasma cell disorder that is not accounted for in this table. This entity should be
§
considered separately, A plasma VEGF level of 200 pg/mL is 95% specific and 68% REFERENCES
sensitive for a POEMS syndrome, **Because of the high prevalence of diabetes
[12]
mellitus and thyroid abnormalities, this diagnosis alone is not sufficient to meet this 1. Crow RS. Peripheral neuritis in myelomatosis. Br Med J
minor criterion, ***Approximately 50% of patients will have bone marrow changes that
distinguish it from a typical monoclonal gammopathy of undetermined significance or 1956;2:802‑4.
myeloma bone marrow. [24] POEMS: polyneuropathy, organomegaly, endocrinopathy, 2. Nakanishi T, Sobue I, Toyokura Y, Nishitani H, Kuroiwa Y,
M proteins and skin changes, VEGF: Vascular endothelial growth factor Satoyoshi E, Tsubaki T, Igata A, Ozaki Y. The Crow‑Fukase syndrome:
42 Neuroimmunol Neuroinflammation | Volume 1 | Issue 1 | June 2014