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4FI conference

4FI conference. A 58y/o male patient with POEMS syndrome. Ri 周柏安 /VS 郭順文. Brief history(1). 58y/o male, with DM under OHA BPH Hypertension POEMS syndrome, first diagnosed in 1994/10, controlled under regular OPD follow-up. Brief history(2). Progression of POEMS syndrome

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4FI conference

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  1. 4FI conference A 58y/o male patient with POEMS syndrome Ri周柏安/VS郭順文

  2. Brief history(1) 58y/o male, with • DM under OHA • BPH • Hypertension • POEMS syndrome, first diagnosed in 1994/10, controlled under regular OPD follow-up

  3. Brief history(2) Progression of POEMS syndrome • initial manifestation of weakness and numbness of distal parts of four limbs • Receive regular OPD follow-up︰ admitted to 5D three times, had tried chemotherapy and plasma exchange therapy 3. In 2005/12, condition worsening︰ increasing dysesthesia & rigidity, poor appetite, dizziness, nausea, vomiting after food intake, and abdominal fullness

  4. Brief history(3) 2006/01 CT︰ gallstones, ascites, and left renal cysts, Panendoscopy︰ GERD & congestive gastropathy Abdominal echo ︰ suspect liver cirrhosis & ascites paracentesis*2 2006/05/16︰ admitted to 12D for rapid deterioration of neuropathy

  5. Brief history(4) In 5D︰ NCV and SSEP︰degenerative axonopathy Serum eletrophoresis ︰IgG:1310 mg/dl, IgA:320 mg/dl, IgM:77 mg/dl , no monoclonal protein Ascites tapping︰1000 mL clear yellowish fluid , albumin of 2.3 g/dL, LDH of 122 mg/dL, glucose of 115 mg/dL CSF protein level︰120 mg/dL plasmapheresis*7 muscle power increased significantly

  6. Brief history(5) In 5D, abdominal echo was performed hepatosplenomegaly with massive ascites Laparoscopic wedge resection of liver on 2006/6/2 op findings ︰ • Massive ascites, ~2500ml, yellow & clear • liver ︰graish-whitish change 3. Reddish fibrin like substance over abdomen cavity

  7. Brief history(6) Post-op in ICU ︰ Hemodynamic unstable, Hb ︰11.1 on 6/1, 7.8 on 6/3 WBC↑, Plt↓ continuous blood transfusion Hb, Plt still drop after PRBC, FFP usage; WBC level still higher, abdominal fullness occurred exploratory laparotomy was performed on 06/05 massive ascites 3600cc and diffuse oozing around LUQ port site without any active bleeder.

  8. Brief history(7) Post-op in 4FI ︰ Vital sign ︰ BT:36.2, BP:144/89, PR:120 Respiratory ︰ 1ETT intubation , under ventilator support -- Mode: SIMV+PS(8) , RR: 21/8 Vt:554 PEEP :5  Easily dyspnea and irregular respiration while weaning

  9. Brief history(8) Hematology study -- Hb: 13.2 (R-D︰amount in descent, exsudative in nature) -- INR: 1.58 -- Alb:3.23 -- WBC:18260 (Rocephin since 6/5, Tazocin since 6/9) -- CRP:5.48 -- BUN: 60,

  10. Brief history(9) 6/8 dyspnea CxR:minimal left side pleural effusion, cardiac echo: LVEF:30.1% , no significant hypertrophy or dilatation plasmapheresis is hold due to high risk  CV man suggest dopamine usageLVEF improve to 60% -->RT : Abdominal weakness must be treated for successful weaning dr. 謝: no significant involvement of phrenic nerve

  11. Brief history(10) Other problems: • Poor NG feedingPrimperan + Dulcolax, TPN since 6/9 • Positive I/O for several dayslasix+aldactone also check R/D for few ascites was drained 3. Hypernatremia(CVP :6-9cm)may due to lasix usage, because: urine osm:606mosm/Kg, 24hr urine amount:1450ml

  12. POEMS syndrome

  13. Introduction Also named as osteosclerotic myeloma Named after • Polyneuropathy • Organomegaly • Endocinopathy • Monoclonal protein • Skin change

  14. Pathophysiology • Cause unknown, may due to overproduction of the proinflammatory and other cytokines, especially VEGF • Cytokines higher in POEMS syndrome than in multiple myeloma︰ IL-1β, TNF- α, IL-6； lower ︰TGF-1β • IL-6 ︰indicator of disease severity Ps: Age & sex factor—mean age: 51y/o, 63% is male

  15. Clinical feature(1) Peripheral neuropathy • Progressive and usually dominate the clinical picture • from distal to proximal, but cranial nerves are not affected；motor involvement follows sensory symptoms • Autonomic symptoms are not a feature • Severe weakness occurred in 1/2～ • Combines axonal degeneration and demyelination

  16. Clinical feature(2) Organomegaly︰ • hepatomegaly(70%), splenomegaly(40%), lymphadenopathy(55%) • Enlargement are often mild • Liver biopsy often reveals non-specific change • Biopsy of enlarged lymph nodeeither Castleman’s disease or reactive change

  17. Clinical feature(3) Endocine abnormalities︰ • Two –thirds of patients have at least one endocrine abnormality • Hypogonadism is the most common male︰ impotency, gynecomastia female︰ galactorrhea 3. others︰ hypothyroidism, adrenal insufficiency, DM, hyperparathyroidism

  18. Clinical feature(4) Monoclonal plasma cell disorder • Magnitude of serum monoclonal protein was small, mean M-spike︰ 1.1g/dL • The type of light chain in POEMS syndrome is almost always λ 3. Besides serum, M-protein may also be found in urine & CSF

  19. Clinical feature(5) Skin change︰ hyperpigmentation, hypertrichosis, acrocyanosis, plethora, hemangioma/telangiectasia Bone lesion ︰ • Can be sclerotic or mixed with lytic lesions, purely lytic is rare • May be small in size, d/d with benign bony sclerosis Renal disease Cardiovascular disease ︰ Thrombotic event, extravascular volume overload, peripheral edema, ascites, pleural effusion, pericarditis

  20. Clinical feature(5) Pulmonary involvement︰ pulmonary hypertension, constrictive lung disease, pleural effusion, respiratory failure, clubbing of the finger Hematologic features ︰RBC↑ , WBC↑ , Plt↑ , bone marrow examination is most often nondiagnostic Other symptoms and signs ︰ 1. increased CSF protein 2. BW loss(>10 pounds) 3. fatigue 4. Papillary edema 5.edema, ascites, pleural effusion 6.clubbing

  21. Diagnosis—Mayo clinic criteria

  22. Therapy • Radiation therapy ---a. single or multiple osteosclerotic within limited area b. dose︰ 40-50Gy • Chemotherapy Mephalan & prednisolone, …… • Hematopoietic cell transplantation ---a. stem cell should be collected before chemo.

  23. Thanks for your attention!

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