Page 1124 - Clinical Immunology_ Principles and Practice ( PDFDrive )

1088 Part eight Immunology of Neoplasia

and absence of anemia, hypercalcemia, or renal insufficiency TABLE 80.4 iMWg Diagnostic Criteria for
attributable to a clonal plasma cell proliferative disorder. 16 the Diagnosis of POeMS Syndrome
Some patients may have a small amount of M-protein, usually
IgA, in the serum or urine, which often disappears after treatment. Both Mandatory Criteria:
The bone marrow of patients with SEP does not have clonal 1. Polyneuropathy
plasma cells, whereas some patients with a solitary extramedullary 2. Monoclonal plasma cell proliferative disorder
lesion may demonstrate up to 10% clonal plasma cells, in which Plus at Least One Major Criterion:
case the diagnosis is SEP with minimal marrow involvement. 15 3. Osteosclerotic or mixed sclerotic/lytic lesion visualized on plain
films or computed tomography (CT)
Management and Prognosis 4. Castleman disease
The treatment of choice for SEP is RT. Small lesions may be 5. Elevated serum or plasma vascular endothelial growth factor
cured with surgery alone; no adjuvant RT is indicated unless (VEGF) levels (at least three to four times the upper limit of
there is suspicion of residual local disease. 15 normal)
Less than 7% of patients with SEP will develop a local recur- Plus at Least One Minor Criterion:
rence after tumoricidal radiation. Approximately 10–15% of
patients ultimately will develop MM; the progression rate is 1. Organomegaly (splenomegaly, hepatomegaly, or
lymphadenopathy)
higher (20%) in patients with SEP with minimal marrow involve- 2. Extravascular volume overload (peripheral edema, ascites, or
ment. Five-year overall survival rates range from 40–85%. 15 pleural effusion)
3. Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, or
pancreatic disorder excluding diabetes mellitus or hypothyroidism)
POEMS SYNDROME 4. Skin changes (hyperpigmentation, hypertrichosis, glomeruloid
hemangiomata, plethora, acrocyanosis, flushing, white nails)
POEMS (polyneuropathy, organomegaly, endocrinopathy, 5. Papilledema
monoclonal protein, skin changes) syndrome is characterized 6. Thrombocytosis or polycythemia
by the presence of a monoclonal plasma cell disorder, peripheral
neuropathy, and one or more of the following features: osteo- POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin
sclerotic myeloma, Castleman disease, increased levels of serum changes.
Source: Rajkumar SV, Dimopoulos MA, Palumbo A, Blade J, Merlini G, Mateos MV,
vascular endothelial growth factor (VEGF), organomegaly, et al. International Myeloma Working Group updated criteria for the diagnosis of
endocrinopathy, edema, typical skin changes, and papilledema. multiple myeloma. Lancet Oncol 2014;15(12):e538–48. doi: 10.1016/S1470-
The cause of POEMS syndrome is unknown, although chronic 2045(14)70442-5. PubMed PMID: 25439696.
overproduction of proinflammatory and other cytokines such
as VEGF appears to be a major feature of this disorder. POEMS
syndrome is a rare disorder, but the exact incidence is unknown.
POEMS syndrome commonly presents in the fifth to sixth macytic cells and a monoclonal IgM gammopathy in the blood.
decade. 17 WM is a rare disorder with an incidence of approximately 3/
million people/year, with 1400 new cases diagnosed in the United
Clinical Presentation States each year. The median age at diagnosis is 64 years; less
By definition, all patients have peripheral neuropathy and a than 1% of patients are diagnosed under 40 years of age, and
monoclonal plasma cell disorder, almost always of the lambda approximately 60% are males. WM is more common in Caucasians
light chain type. In addition, almost all patients have osteosclerotic than in other ethnic groups, and familial predisposition is present
lesions, whereas Castleman disease is present in approximately in up to 20%. 18
15%. 17
Clinical Presentation
Diagnosis Patients usually present in their seventh decade with symptoms
The IMWG has developed a series of criteria required for the related to the infiltration of the hematopoietic tissues or to the
diagnosis of POEMS syndrome; other signs and symptoms that effects of monoclonal IgM in the blood. WM most commonly
do not fall into these criteria may also be present, as outlined presents with pallor, oronasal bleeding, systemic complaints
in Table 80.4. 8 (weakness, fatigue, weight loss, fever, night sweats), and organo-
megaly (enlarged lymph nodes, spleen, and/or liver). Involvement
Management and Prognosis of the bone or kidneys is uncommon. 18
There is no standard treatment for POEMS syndrome. Those An important presentation includes central nervous system
with limited disease may be treated with RT. Patients with signs and symptoms due to the hyperviscosity syndrome (blurring
widespread bone lesions may be treated with therapy similar to or loss of vision, headache, ataxia, dementia, stroke, or coma).
17
that used for MM. POEMS syndrome is chronic, and patients A classic finding in WM associated with hyperviscosity is the
with POEMS syndrome survive three times longer than those presence of dilated, segmented, and tortuous retinal veins. Another
with MM. The natural history is one of progressive peripheral major presentation involves neurological symptoms such as
neuropathy until the patient is bedridden. Death usually occurs paresthesias and weakness as well as cranial nerve palsies and
from inanition or a terminal bronchopneumonia. 17 sudden deafness. Patients with WM who are asymptomatic are
considered to have SWM. 18
WALDENSTRÖM MACROGLOBULINEMIA Laboratory Findings and Diagnosis

WM is a rare clinicopathological entity demonstrating 10% or Patients with WM must have 10% or greater infiltration of the
greater infiltration of the bone marrow by clonal lymphoplas- bone marrow by clonal lymphoplasmacytic cells and a monoclonal