Nezam Torok , MD
PGY2

History :
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A 42 year old African American Man
CC : Back pain , for 1 week

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Nov 2008 : Pericardial effusion
Dec 2008 : Suspecion of sarcoidosis
PMH :

Social history
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Smoker 30 PPY
 sexual dysfunction

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Neurological : Motor function : power 4/5 lower limb –leg flexor and extensors as well as foot , bilaterally with diminished DTR and decreased sensation to touch , pain , and position , otherwise negative .

More lab tests :
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HIV , HHV 8 negative
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Normal or negative tests include : TFT ,
LH , FSH , testosterone ,ANA , ANCA ,
ACE ,RF , SS-A , SS-B , C3, C4, Hep B ,
Hep C ,PSA, vit B12 , APA .

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SPE :
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 increase in IgG 1950 mg/dL(normal 590-1540 mg/dL) mild increase in IgA 498 (normal 50-413mg/dL ) serum immunofixation polyclonal hypergammaglobulinemia with slightly excess lambda chains , kappa chains 1040 mg/dL ( normal 539 -1320 mg/dL) , and Lambda chains
690mg/dL (normal 285-673mg/dL ) with kappa /lambda ratio of 1,5 .

Normal architecture

Effaced architecture of the LN in castlemans disease , HE stain 4 X
Normal architecture of LN

Atrophic germinal centers
, HE stain 40X
Normal germinal center, containing larger lymphocytes undergoing activation

Atrophic germinal center and effaced architercture

Vascular proliferation ,10 X

Vascular proliferation 20 X

Vascular proliferation 40 X

 interleukin 6 level: 6 pg/ml (normal 0-5pg/ml)
 plasma Vascular Endothelial Growth factor level
(VEGF): 130 pg/ml (normal 6-86 pg/ml)
 normal interleukin 1.

Summary of findings
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Osteosclerotic lesions
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Neuropathy motor and sensory
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Skin changes
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Lymphadenopathy
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History of pericardial effusion
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Castlemans disease
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High IL-6 and VEGF

Multicentric Castleman’s disease with features of
POEMS syndrome (Castle-
POES)!

Treatment
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There is no standard treatment for this disorder and no randomized or quasi-randomized controlled clinical trials of treatment for POEMS syndrome exist in the available literature*
* Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, Mprotein, and skin changes) syndrome. Kuwabara S; Dispenzieri A; Arimura K;
Misawa S Cochrane Database Syst Rev. 2008 Oct 8;(4):CD006828

How did we treat the patient
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How did the patient respond
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Remarkable improvement in muscle power , able to function again
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Fatigue resolved
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Sexual function improved.
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Dec PET scan activity at the scerotic bone lesions
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No lymphadenopathy

BUT
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VEGF was still at the same level .
 no change in skin lesions.
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SPEP and immunofixation the same pattern

differential scanning calorimeter (DSC)

differential scanning calorimeter
(DSC
Our Patient
Control

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The DST shows abnormal thermogram, which means there is abnomal molecular species present in the serum of the serum sample that interact in a significant way with one or more of the proteins in the serum
 it is different from electrophoresis technique that are sensitive to size and charge
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What kind of protein , whats is the relation to immunofixation ?

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“ Thinking is more interesting than knowing, but less interesting than looking
”.

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References
Bradwick PA, Zvaifler NJ, Gill GN, et al. Plasma celldyscrasia with polyneuropathy, organomegaly,endocrinopathy, M protein and skin changes: the POEMSsyndrome.
Medicine 1980; 59: 311–322
Dispenzieri A, Kyle RA, Lacy MQ, et al. POEMS syndrome: definitions and long-term outcome. Blood 2003;101:2496–506
Nakanishi T, Sobue I, Toyokura Y, et al. The Crow-Fukase syndrome: a study of 102 cases in Japan. Neurology 1984;34:712-20.
Shikama N, Isono A, Otsuka Y, Terano T, Hirai A .a case of POEMS syndrome with high concentration of IL-6 in pericardial fluid .
J Intern Med. 2001 Aug;250(2):170-3
Hashiguchi T, Arimura K, Matsumuro K, et al. Highly concentrated vascular endothelial growth factor in plateletsin Crow-Fukase syndrome. Muscle Nerve.
2000;23:1051-1056.
Soubrier M, Dubost JJ, Serre AF, et al. Growth factors in POEMS syndrome: evidence for a marked increase in circulating vascular endothelial growth factor. Arthritis
Rheum. 1997;40:786-787
Joanne Shirine Allam, Cassie C. Kennedy, Timothy R. Aksamit and Angela Dispenzieri
. Pulmonary Manifestations in Patients With POEMS Syndrome. Chest 2008;133;969-
974