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Castle-POES disease successfully treated with Rituximab

Castle-POES disease successfully treated with Rituximab. Nezam Torok , MD PGY2. History : . A 42 year old African American Man CC : Back pain , for 1 week . PMH : . Nov 2008 : Pericardial effusion Dec 2008 : Suspecion of sarcoidosis. Social history . Smoker 30 PPY

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Castle-POES disease successfully treated with Rituximab

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  1. Castle-POES disease successfully treated with Rituximab Nezam Torok , MD PGY2

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

  3. PMH : • Nov 2008 : Pericardial effusion • Dec 2008 : Suspecion of sarcoidosis

  4. Social history Smoker 30 PPY sexual dysfunction

  5. 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 .

  6. More lab tests : HIV , HHV 8 negative 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 .

  7. SPE : • 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 .

  8. Normal architecture

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

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

  11. Atrophic germinal center and effaced architercture

  12. Vascular proliferation ,10 X

  13. Vascular proliferation 20 X

  14. Vascular proliferation 40 X

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

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

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

  18. Treatment • 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, M-protein, and skin changes) syndrome. Kuwabara S; Dispenzieri A; Arimura K; Misawa S Cochrane Database Syst Rev. 2008 Oct 8;(4):CD006828

  19. How did we treat the patient Rituximab 375/m2 , weekly for 4 weeks , then once every 2-3 months

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

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

  22. Investigational model differential scanning calorimeter (DSC)

  23. differential scanning calorimeter (DSC Our Patient Control

  24. 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 What kind of protein , whats is the relation to immunofixation ?

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

  26. 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 ShirineAllam, Cassie C. Kennedy, Timothy R. Aksamit and Angela Dispenzieri. Pulmonary Manifestations in Patients With POEMS Syndrome. Chest 2008;133;969-974

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