A Case of POEMS Syndrome

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A Case of POEMS Syndrome

  1. 1. PROF.DR.NOORUL AMEEN PANDICHELVAN M6
  2. 2. <ul><li>40 yrs old female came with </li></ul><ul><li>c/o breathlessness -2 mon </li></ul><ul><li>insidious onset </li></ul><ul><li>progressive </li></ul><ul><li>class 2 </li></ul><ul><li>related with exertion </li></ul><ul><li>h/o easy fatigability </li></ul><ul><li>h/o arthralgia </li></ul><ul><li>h/o tiredness </li></ul><ul><li>h/o loss of weight </li></ul>
  3. 3. <ul><li>No h/o cough and sputum production </li></ul><ul><li>palpitation </li></ul><ul><li>orthopnea </li></ul><ul><li>paroxysmal nocturnal dyspnea </li></ul><ul><li>chest pain </li></ul><ul><li>Past history: </li></ul><ul><li>Not know case of DM/HT/PT </li></ul><ul><li>Personal history: </li></ul><ul><li>Not an Alcoholic/Smoker </li></ul><ul><li>Menstrual history: </li></ul><ul><li>Attained menarche @ 12 yrs of age </li></ul><ul><li>Regular; once in 26 days </li></ul><ul><li>Last for 4 days </li></ul>
  4. 5. <ul><li>General examination </li></ul><ul><li>Conscious </li></ul><ul><li>Oriented </li></ul><ul><li>Pallor </li></ul><ul><li>Hyperpigmentation seen </li></ul><ul><li>Scar seen in left side neck </li></ul><ul><li>Not Icteric </li></ul><ul><li>No Cyanosis </li></ul><ul><li>No Clubbing </li></ul><ul><li>No Pedal edema </li></ul><ul><li>No Lymph node enlargement </li></ul><ul><li>BP 90/60 mmhg Pulse rate 70/min </li></ul>
  5. 6. <ul><li>CVS : S1S2 heard </li></ul><ul><li>P2 loud </li></ul><ul><li>RS : NVBS </li></ul><ul><li>BAE </li></ul><ul><li>No added sound </li></ul><ul><li>P/A: Soft </li></ul><ul><li>CNS: NFND </li></ul>
  6. 7. <ul><li>Provisional diagnosis </li></ul><ul><li>Pulmonary hypertension </li></ul><ul><li>? Addison disease </li></ul><ul><li>? MCTD </li></ul>
  7. 8. <ul><li>INVESTIGATION </li></ul><ul><li>Urine examination </li></ul><ul><li>Albumin- nil </li></ul><ul><li>Sugar - nil </li></ul><ul><li>Deposit- nil </li></ul><ul><li>CBC </li></ul><ul><li>Hb -9.2 TC -5700 </li></ul><ul><li>DC - P60 L40 E0 PCV -33 </li></ul><ul><li>ESR -6/15 PLC -1.6 lakh </li></ul><ul><li>MCV -86 </li></ul><ul><li>BLOOD Urea 36 </li></ul><ul><li>Sugar 106 </li></ul><ul><li>Creatinine 0.8 </li></ul><ul><li>Sodium 130 </li></ul><ul><li>Potasium 3.9 </li></ul>
  8. 9. <ul><li>ECG –normal </li></ul><ul><li>CXR - Cardiomegaly </li></ul>
  9. 10. <ul><li>Echocardiography </li></ul>
  10. 14. <ul><li>BLOOD IMMUNOLOGY </li></ul><ul><li>ANA Positive 1:40 dilution </li></ul><ul><li>CRP Negative </li></ul><ul><li>ASO Negative </li></ul><ul><li>RF Negative </li></ul>
  11. 15. <ul><li>Hypothyroidism </li></ul><ul><li>Pulmonary hypertension </li></ul><ul><li>Anemia </li></ul><ul><li>? Cause </li></ul>
  12. 16. <ul><li>Patient treated with </li></ul><ul><li>Tab. Thyroxine 100 ug od </li></ul><ul><li>Tab. Deriphylline tds </li></ul><ul><li>Tab. Bct tds </li></ul><ul><li>Patient advised to do </li></ul><ul><li>ACTH stimulation test </li></ul>
  13. 17. <ul><li>After two months patient comes with </li></ul><ul><li>c/o breathlessness </li></ul><ul><li>c/o abdominal pain </li></ul><ul><li>c/o athralgia </li></ul><ul><li>c/o numbness in both lower limbs </li></ul><ul><li>c/o hard of hearing </li></ul>
  14. 18. <ul><li>On examination </li></ul><ul><li>Conscious </li></ul><ul><li>Oriented </li></ul><ul><li>Pallor </li></ul><ul><li>Not Icteric </li></ul><ul><li>No Cyanosis </li></ul><ul><li>No Clubbing </li></ul><ul><li>B / L Pitting pedal edema </li></ul><ul><li>No Lymphnode enlargement </li></ul>
  15. 19. <ul><li>CVS: S1S2 heard </li></ul><ul><li>P2 loud </li></ul><ul><li>RS: NVBS </li></ul><ul><li>BAE </li></ul><ul><li>No Added sound </li></ul><ul><li>P/A: Abdomen distension </li></ul><ul><li>Moves with respiration </li></ul><ul><li>Flanks full </li></ul><ul><li>Shifting dullness + </li></ul>
  16. 20. <ul><li>CNS: HMF Normal </li></ul><ul><li>CN Normal </li></ul><ul><li>TONE Normal </li></ul><ul><li>POWER RT LT </li></ul><ul><li>UL 4 4 </li></ul><ul><li>LL 4 4 </li></ul><ul><li>DTR Present Present </li></ul><ul><li>B/L Plantar Flexor </li></ul><ul><li>Sensory system – Vibration sensation lost in both lower limbs </li></ul><ul><li>Cerebellar and autonomic nervous system- Normal </li></ul>
  17. 21. <ul><li>Hypothyroidism </li></ul><ul><li>Pulmonary hypertension </li></ul><ul><li>Ascities </li></ul><ul><li>Periperal neuropathy </li></ul><ul><li>Anemia </li></ul><ul><li>Cause - ? MCTD </li></ul><ul><li>? Autoimmune polyglandular syndrome </li></ul>
  18. 22. <ul><li>INVESTIGATIONS </li></ul><ul><li>Urine Examinations </li></ul><ul><li>Albumin-1+ </li></ul><ul><li>Sugar-nil </li></ul><ul><li>Deposit – 4 to 5 pus cells/hpf </li></ul><ul><li>Renal lab values </li></ul><ul><li>Glucose – ve </li></ul><ul><li>Bilirubin neg </li></ul><ul><li>Ketone neg </li></ul><ul><li>Specific gravity 1.010 </li></ul><ul><li>Blood 2+ </li></ul><ul><li>ph 5.0 </li></ul><ul><li>Protein 2+ </li></ul><ul><li>Nitrogen neg </li></ul><ul><li>Leucocytes neg </li></ul>
  19. 23. <ul><li>24 hr urine protein- 20mg/day </li></ul><ul><li>COMPLETE BLOOD HAEMOGRAM </li></ul><ul><li>HB-11.5 </li></ul><ul><li>RBC-3MILLION/CUMM </li></ul><ul><li>ESR-10/22 </li></ul><ul><li>MCHC-30.1 </li></ul><ul><li>MCV-79.4 </li></ul><ul><li>MCH-28.4 </li></ul><ul><li>PCV-31 </li></ul><ul><li>PLT-1.2 </li></ul><ul><li>TC-5200 </li></ul><ul><li>DC-P69;L30;E1 </li></ul>
  20. 24. <ul><li>BLOOD Sugar-105mg FBS-59MG/DL; PPBS-123MG/DL </li></ul><ul><li>Urea-71mg </li></ul><ul><li>Creatinine-1.5mg </li></ul><ul><li>Sodium-135 meq/l </li></ul><ul><li>Potasium-5.1meq/l </li></ul><ul><li>Chloride-108 meq/l </li></ul><ul><li>LIVER FUNCTION TEST </li></ul><ul><li>Total bilirubin-0.8 </li></ul><ul><li>SGOT-18 </li></ul><ul><li>SGPT-16 </li></ul><ul><li>ALP-78 </li></ul><ul><li>Total protein-5.9 </li></ul><ul><li>Albumin-3.9 </li></ul>
  21. 25. <ul><li>ABG ANALYSIS : ph-7.38 </li></ul><ul><li>po2-85.4mmhg </li></ul><ul><li>pco2-27.6mmhg </li></ul><ul><li>hco3-16.2mmol/l </li></ul><ul><li>h+-41.2nmol/l </li></ul><ul><li>na-131.3mmol/l </li></ul><ul><li>k-5.50mmol/l </li></ul><ul><li>ag-18.9mmol/l </li></ul><ul><li>Absolute eosinophil count-80 cells/cumm </li></ul>
  22. 26. ASCITIC FLUID ANALYSIS Protein-4.4 Sugar-92 Aalbumin-1.2 SAAG -2.66 Cell count - 96%lymphocytes;4%mesothelial cells= Cytology- clusters of reactive mesothelial cells and few lymphocytes in a eosinophilic background-reactive effusion Mantouex test : Negative
  23. 27. <ul><li>USG ABDOMEN </li></ul><ul><li>Ascities </li></ul><ul><li>B/L increased renal cortical echos </li></ul><ul><li>Liver – normal size </li></ul><ul><li>Spleen- normal size </li></ul><ul><li>ECHO- </li></ul><ul><li>RA/RV dilated </li></ul><ul><li>TR moderate pg 46mmhg </li></ul><ul><li>Moderate pulmonary hypertension </li></ul><ul><li>No RWMA </li></ul><ul><li>Normal LT ven systolic function </li></ul>
  24. 29. <ul><li>ENDOCRINOLOGY STUDY </li></ul><ul><li>THYROID FUNCTION TEST </li></ul><ul><li>T3- 47.02ng/dl(80-200ng/dl) </li></ul><ul><li>T4- 5.77ugm/dl(4.6-12ugm/dl) </li></ul><ul><li>TSH-16.322miu/ml(0.27-4.20miu/ml) </li></ul><ul><li>Anti TPO antibodies -30.39 iu/ml(0-34iu/ml) </li></ul><ul><li>Serum Cortisol -8.32ugm/dl(6.2-19.4iu/ml) </li></ul><ul><li>ACTH stimulation test </li></ul><ul><li>serum Cortisol-8.53ugm/dl </li></ul><ul><li>DHEA sulphate-21.60ugm/dl(80-560ugm/dl) </li></ul><ul><li>Parathyroid hormone – 77.82pg/ml (15-65pg/ml) </li></ul>
  25. 30. <ul><li>Patient treated with </li></ul><ul><li>Tab.Prednisolone 5mg 6 tablets </li></ul><ul><li>Tab.Fludrocortisone 50ugm </li></ul><ul><li>Cap.Omeprazole 40mg bd </li></ul><ul><li>Tab.Eltroxine 100 mg bd </li></ul><ul><li>Tab. Calcium lactate tds </li></ul>
  26. 32. <ul><li>BLOOD IMMUNOLOGY </li></ul><ul><li>ANA- Positive 1:100 dilution </li></ul><ul><li>Ds-DNA ab Negative </li></ul><ul><li>Scl 70 ab Negative </li></ul><ul><li>Anti centromere ab Negative </li></ul><ul><li>Sm antibody Negative </li></ul><ul><li>U1 nrnp Negative </li></ul><ul><li>Blood HBsAG :Negative </li></ul><ul><li>Anti hcv: Negative </li></ul><ul><li>hiv elisa:Negative </li></ul><ul><li>vdrl Negative </li></ul>
  27. 33. <ul><li>Pulmonary </li></ul><ul><li>function </li></ul><ul><li>test </li></ul>
  28. 36. <ul><li>Ct abdomen </li></ul>
  29. 43. <ul><li>Blood biochemistry </li></ul><ul><li>Calcium-11.1 mg/dl then comes to 8.6 mg /dl </li></ul><ul><li>Phosphorus -5.1mg/dl </li></ul><ul><li>LDH-131u/l </li></ul><ul><li>ALP-104.1 iu/l </li></ul><ul><li>24 hr urine calcium 56mg/dl </li></ul>
  30. 44. Differential diagnosis for metastatic sclerotic leisons <ul><li>Thyroid carcinoma </li></ul><ul><li>Renal cell carcinoma </li></ul><ul><li>Breast carcinoma </li></ul><ul><li>Gastric carcinoma </li></ul><ul><li>Skin carcinoma </li></ul>
  31. 49. <ul><li>Nerve conduction study </li></ul>
  32. 52. <ul><li>Results: </li></ul><ul><li>Upper limb </li></ul><ul><li>Right median and ulnar nerve CMAP latency,amplitude and velocity –WNL </li></ul><ul><li>Left side - Absent </li></ul><ul><li>Lower limb </li></ul><ul><li>both CMAP AND SNAP not obtained </li></ul><ul><li>Impression </li></ul><ul><li>Both sensory motor neuropathy of both lower limb and possible sensory motor neuropathy of upper limb </li></ul>
  33. 53. <ul><li>Meanwhile patient said </li></ul><ul><li>c/o headache –B/L </li></ul><ul><li>Diffuse </li></ul><ul><li>Dullache </li></ul>
  34. 55. <ul><li>Patient developed lymphnode enlargement </li></ul><ul><li>B/L inguinal </li></ul><ul><li>B/L axillary </li></ul><ul><li>FNAC </li></ul><ul><li>Rt inguinal LN: c/c lymphadenitis </li></ul><ul><li>Left inguinal LN:material inadequate for definite opinion </li></ul><ul><li>Right axilla LN: granulomatous lymphadenitis </li></ul><ul><li>Left axilla LN: c/c lymphadenitis </li></ul>
  35. 56. <ul><li>biopsy </li></ul>
  36. 58. <ul><li>Serum electrophoresis- normal </li></ul><ul><li>protein 5.1 </li></ul><ul><li>albumin 3.2 </li></ul><ul><li>Urine BJP Negative </li></ul><ul><li>Serum ACE 16.3 u/l(8-52) </li></ul>
  37. 65. <ul><li>Patients have </li></ul><ul><li>hypothyroidism </li></ul><ul><li>addison disease </li></ul><ul><li>hyperparathyroidism </li></ul><ul><li>pht </li></ul><ul><li>severe restrictive lung disease </li></ul><ul><li>papilledema </li></ul><ul><li>glomerulonephritis </li></ul><ul><li>ascities </li></ul><ul><li>b/l pitting pedal edema </li></ul><ul><li>polyneuropathy </li></ul><ul><li>m protein elevation </li></ul><ul><li>sensory neural hearing loss </li></ul><ul><li>multipe osteosclerotic leison </li></ul><ul><li>castle man disease-multicentric </li></ul><ul><li>Clinically diagnosed as poems syndrome </li></ul>
  38. 66. <ul><li>Haematology opinion: </li></ul>
  39. 71. <ul><li>Final diagnosis </li></ul><ul><li>POEMS SYNDROME </li></ul><ul><li>P- polyneuropathy </li></ul><ul><li>O-organomegaly </li></ul><ul><li>E-endocrinopathy </li></ul><ul><li>M-m protein component </li></ul><ul><li>S- skin changes </li></ul>
  40. 72. <ul><li>Patient treated with </li></ul><ul><li>Tab.Prednisolone 5 mg 6 tab </li></ul><ul><li>Tab.Fludrocortisone 50 ug </li></ul><ul><li>Tab.Eltroxine 100 ug od </li></ul><ul><li>Tab.Amitriptylline 2.5 mg bd </li></ul><ul><li>Tab.Calcium lactate tds </li></ul><ul><li>Cap.Omeprazole 40 mg bd </li></ul>
  41. 73. Poems syndrome <ul><li>Defined as the combination of plasma cell proliferation disorder(typically myeloma) , polyneuropathy and effects on many other organ systems </li></ul><ul><li>Peak incidence around 5-6 th decade of life unlike myeloma which is common around 6-7 th decade of life </li></ul><ul><li>Twice as common in men </li></ul><ul><li>Other names: osteo sclerotic myeloma, pep syndrome, crow fukase syndrome, takatsuki syndrome </li></ul>
  42. 74. <ul><li>DIAGNOSTIC CRITERIA </li></ul><ul><li>Polyneuropathy </li></ul><ul><li>Monoclonal plasma cell disorder </li></ul><ul><li>One or more of the following major criteria </li></ul><ul><li>- Sclerotic bone lesion </li></ul><ul><li>- Castleman’s disease </li></ul><ul><li>- Elevated VEGF levels </li></ul><ul><li>One or more of the following minor criteria </li></ul><ul><li>Organomegaly ( enlarged spleen,liver or lymphnode) </li></ul><ul><li>Extra vascular volume overload(pleural effusion,ascities,pedal edema) endocrinopathy(adrenal,pitutary,gonadal,parathyroid)dm,hypothyroidism alone insufficient </li></ul><ul><li>Papilledema </li></ul><ul><li>Thrombocytosis or Polycythemia </li></ul><ul><li>Skin changes-hyperpigmentation,dermis thickening,hirsutism,hyperhidrosis </li></ul><ul><li>Glomerulonepritis </li></ul><ul><li>Sensory neural hearing loss </li></ul>
  43. 75. <ul><li>These patients have only 5% or less than bone marrow plasma cell </li></ul><ul><li>Rarely have hypercalcemia or renal insufficiency </li></ul><ul><li>1/3 rd to1/2 of pt have poly neuropathy where as in classic multiple myeloma only 1-8% of pts have polyneuropathy </li></ul>
  44. 76. <ul><li>Etiology </li></ul><ul><li>Caue is unknow </li></ul><ul><li>Anti body to HHV 8 seen 78% of cases with castleman disease and 22% of patients without castleman disease </li></ul><ul><li>Increased level VEGF, IL1BETA, IL6 and TNF alpha </li></ul><ul><li>VEGF targets the endothelial cells and promote angiogenesis </li></ul><ul><li>Both IL 1 beta and IL6 stimulate VEGF production </li></ul><ul><li>Elevated level of VEGF correlate with disease activity </li></ul><ul><li>Elevated VEGF accounts for organomegaly,edema and skin changes; but role in polyneuropathy uncertain </li></ul>
  45. 77. <ul><li>POLYNEUROPATHY </li></ul><ul><li>Though resembles CIDP it is motor predominance </li></ul><ul><li>Marked slowing of conduction velocity with prolonged distal latencies and progressive dispersions of compound muscle action potentials with stimulation of motor nerves proximally </li></ul><ul><li>Nerve biopsy shows combination of axonal degeneration and demyelination </li></ul>
  46. 78. <ul><li>ORGANOMEGALY </li></ul><ul><li>Hepatomegaly and Lymphnode enlargement seen in 2/3 rd of patients </li></ul><ul><li>Spleenomegaly seen in 1/3 rd of patients </li></ul><ul><li>Between 11-30% patients have castleman’s disease </li></ul><ul><li>Association is not fully understood </li></ul>
  47. 79. <ul><li>MONOCLONAL PLASMA PROLIFERATIVE DISORDER </li></ul><ul><li>Monoclonal protein is not large </li></ul><ul><li>May be missed on serum electrophoresis in 1/3 rd of patients </li></ul><ul><li>These patients require immunofixation electrophoresis of serum/urine </li></ul><ul><li><10% patients have no circulating protein </li></ul><ul><li>They need biopsy proof of plasmocytoma </li></ul><ul><li>In general number of plasma cells are low and bone marrow shows hypercellular and reported as reactive or myeloproliferative disorder </li></ul>
  48. 80. <ul><li>SCLEROTIC BONE LESION </li></ul><ul><li>Approximately seen in 95% of patients </li></ul><ul><li>One half of patient have solitary sclerotic lesion </li></ul><ul><li>1/3 rd of patient have multiple sclerotic leison </li></ul><ul><li>Pelvis ,spine, ribs and proximal extremites are often involved </li></ul>
  49. 81. <ul><li>PULMONARY DYSFUNCTION </li></ul><ul><li>Pulmonary hypertension,restrictive lung disease and impaired diffusion capacity of carbon monoxide may occur </li></ul><ul><li>Improvement of PHT after therapy has been reported </li></ul><ul><li>Only 19% of poems syndrome initially presented with pulmonary hypertension </li></ul>
  50. 82. <ul><li>HAEMATOLOGY </li></ul><ul><li>50% OF cases have thrombocytosis </li></ul><ul><li>20% of cases have mild erythrocytosis; anemia is not a feature of poems syndrome </li></ul><ul><li>May be arterial or venous thrombosis </li></ul>
  51. 83. <ul><li>TREATMENT </li></ul><ul><li>Corticosteroid-15% patients shows clinical improve ment and 7% of cases shows clinical stabilisation;should not be consider definite therapy rather temporizing therapy </li></ul><ul><li>Alkylating agents </li></ul><ul><li>Cyclophosphamide-as a single agent or in combination with prednisolone shows clinical improvement in 40% of cases </li></ul><ul><li>Melphalan- most effective agent against plasma cell disorder ; but there is risk of mds /acute leukemia </li></ul>
  52. 84. <ul><li>Thalidomide </li></ul><ul><li>Has anti vascular endothelial growth factor and anti tumour necrosis factor </li></ul><ul><li>Cause peripheral neuropathy in 20% patients receiving patients </li></ul><ul><li>Worsen the pedal edema </li></ul><ul><li>As a single agent no more effective than oral alkylators </li></ul>
  53. 85. <ul><li>RADIATION </li></ul><ul><li>patients with single or multiple sclerotic agent in limited area –treated with radiation therapy </li></ul><ul><li>Widespread sclerotic leison –systemic therapy needed </li></ul><ul><li>High dose chemotherapy and stem cell transplantation have been tried </li></ul><ul><li>Intravenous immunoglobulin and plasmapheresis shows no clinical benefit </li></ul><ul><li>Bevacizumab – monoclonal antibody against vegf have been tried </li></ul><ul><li>All trans retenoic acid has been used to suppress the production of proinflammatory cytokines </li></ul>
  54. 86. <ul><li>Prognosis </li></ul><ul><li>If untreated often fatal </li></ul><ul><li>Only 60% of patients alive 5 yrs after onset </li></ul><ul><li>Prognosis is better for patient with single sclerotic leisons </li></ul><ul><li>Multiple bone leison – intermediate prognosis </li></ul><ul><li>Common cause of death-cardiorespiratory failure,infection and capillary leak syndrome </li></ul>
  55. 87. THANK YOU <ul><li>REF: HARRISON 17 EDI </li></ul><ul><li>OTM </li></ul><ul><li>E MEDICINE </li></ul>

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