a case of poems syndrome
Post on 02-Nov-2014
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PROF.DR.NOORUL AMEENPANDICHELVAN
M6
40 yrs old female came with c/o breathlessness -2 mon insidious onset progressive class 2 related with exertion h/o easy fatigability h/o arthralgia h/o tiredness h/o loss of weight
No h/o cough and sputum production palpitation orthopnea paroxysmal nocturnal dyspnea chest painPast history: Not know case of DM/HT/PTPersonal history: Not an Alcoholic/SmokerMenstrual history: Attained menarche @ 12 yrs of age Regular; once in 26 days Last for 4 days
General examination Conscious Oriented Pallor Hyperpigmentation seen Scar seen in left side neck Not Icteric No Cyanosis No Clubbing No Pedal edema No Lymph node enlargement
BP 90/60 mmhg Pulse rate 70/min
CVS : S1S2 heard P2 loud
RS : NVBS BAE No added sound
P/A: Soft
CNS: NFND
Provisional diagnosis Pulmonary hypertension ? Addison disease ? MCTD
INVESTIGATIONUrine examination Albumin- nil Sugar - nil Deposit- nilCBC Hb -9.2 TC -5700 DC - P60 L40 E0 PCV -33 ESR -6/15 PLC -1.6 lakh MCV -86BLOOD Urea 36 Sugar 106 Creatinine 0.8 Sodium 130 Potasium 3.9
ECG –normal
CXR - Cardiomegaly
Echocardiography
BLOOD IMMUNOLOGY ANA Positive 1:40 dilution
CRP Negative
ASO Negative
RF Negative
HypothyroidismPulmonary hypertensionAnemia? Cause
Patient treated withTab. Thyroxine 100 ug odTab. Deriphylline tds Tab. Bct tdsPatient advised to do ACTH stimulation test
After two months patient comes with c/o breathlessnessc/o abdominal painc/o athralgiac/o numbness in both lower limbsc/o hard of hearing
On examination
Conscious Oriented Pallor Not Icteric No Cyanosis No Clubbing B / L Pitting pedal edema No Lymphnode enlargement
CVS: S1S2 heard P2 loud
RS: NVBS BAE No Added sound
P/A: Abdomen distension Moves with respiration Flanks full Shifting dullness +
CNS: HMF Normal CN Normal TONE Normal
POWER RT LT UL 4 4 LL 4 4
DTR Present Present
B/L Plantar Flexor Sensory system – Vibration sensation lost in both lower limbs
Cerebellar and autonomic nervous system- Normal
HypothyroidismPulmonary hypertensionAscitiesPeriperal neuropathyAnemiaCause - ? MCTD ? Autoimmune polyglandular
syndrome
INVESTIGATIONSUrine Examinations Albumin-1+ Sugar-nil Deposit – 4 to 5 pus cells/hpfRenal lab values Glucose – ve Bilirubin neg Ketone neg Specific gravity 1.010 Blood 2+ ph 5.0 Protein 2+ Nitrogen neg Leucocytes neg
24 hr urine protein- 20mg/dayCOMPLETE BLOOD HAEMOGRAM HB-11.5 RBC-3MILLION/CUMM ESR-10/22 MCHC-30.1 MCV-79.4 MCH-28.4 PCV-31 PLT-1.2 TC-5200 DC-P69;L30;E1
BLOOD Sugar-105mg FBS-59MG/DL; PPBS-123MG/DL
Urea-71mg Creatinine-1.5mg Sodium-135 meq/l Potasium-5.1meq/l Chloride-108 meq/l LIVER FUNCTION TEST Total bilirubin-0.8 SGOT-18 SGPT-16 ALP-78 Total protein-5.9 Albumin-3.9
ABG ANALYSIS: ph-7.38 po2-85.4mmhg pco2-27.6mmhg hco3-16.2mmol/l h+-41.2nmol/l na-131.3mmol/l k-5.50mmol/l ag-18.9mmol/l
Absolute eosinophil count-80 cells/cumm
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
USG ABDOMEN Ascities B/L increased renal cortical echos Liver – normal size Spleen- normal sizeECHO- RA/RV dilated TR moderate pg 46mmhg Moderate pulmonary hypertension No RWMA Normal LT ven systolic function
ENDOCRINOLOGY STUDY
THYROID FUNCTION TEST
T3- 47.02ng/dl(80-200ng/dl) T4- 5.77ugm/dl(4.6-12ugm/dl) TSH-16.322miu/ml(0.27-4.20miu/ml)
Anti TPO antibodies -30.39 iu/ml(0-34iu/ml)
Serum Cortisol -8.32ugm/dl(6.2-19.4iu/ml)
ACTH stimulation test serum Cortisol-8.53ugm/dl
DHEA sulphate-21.60ugm/dl(80-560ugm/dl)
Parathyroid hormone – 77.82pg/ml (15-65pg/ml)
Patient treated with Tab.Prednisolone 5mg 6 tablets Tab.Fludrocortisone 50ugm Cap.Omeprazole 40mg bd Tab.Eltroxine 100 mg bd Tab. Calcium lactate tds
BLOOD IMMUNOLOGY ANA- Positive 1:100 dilution Ds-DNA ab Negative Scl 70 ab Negative Anti centromere ab Negative Sm antibody Negative U1 nrnp NegativeBlood HBsAG :Negative Anti hcv: Negative hiv elisa:Negative vdrl Negative
Pulmonary function test
Ct abdomen
Blood biochemistry Calcium-11.1 mg/dl then comes to
8.6 mg /dl Phosphorus -5.1mg/dl LDH-131u/l ALP-104.1 iu/l24 hr urine calcium 56mg/dl
Differential diagnosis for metastatic sclerotic leisonsThyroid carcinomaRenal cell carcinomaBreast carcinomaGastric carcinomaSkin carcinoma
Nerve conduction study
Results:Upper limb Right median and ulnar nerve CMAP
latency,amplitude and velocity –WNL Left side - AbsentLower limb both CMAP AND SNAP not obtainedImpression Both sensory motor neuropathy of both
lower limb and possible sensory motor neuropathy of upper limb
Meanwhile patient said c/o headache –B/L Diffuse Dullache
Patient developed lymphnode enlargement B/L inguinal B/L axillaryFNACRt inguinal LN: c/c lymphadenitisLeft inguinal LN:material inadequate for
definite opinionRight axilla LN: granulomatous lymphadenitisLeft axilla LN: c/c lymphadenitis
biopsy
Serum electrophoresis- normal protein 5.1 albumin 3.2Urine BJP NegativeSerum ACE 16.3 u/l(8-52)
Patients have hypothyroidism addison disease hyperparathyroidism pht severe restrictive lung disease papilledema glomerulonephritis ascities b/l pitting pedal edema polyneuropathy m protein elevation sensory neural hearing loss multipe osteosclerotic leison castle man disease-multicentricClinically diagnosed as poems syndrome
Haematology opinion:
Final diagnosis POEMS SYNDROMEP- polyneuropathyO-organomegalyE-endocrinopathyM-m protein componentS- skin changes
Patient treated with Tab.Prednisolone 5 mg 6 tab Tab.Fludrocortisone 50 ug Tab.Eltroxine 100 ug od Tab.Amitriptylline 2.5 mg bd Tab.Calcium lactate tds Cap.Omeprazole 40 mg bd
Poems syndromeDefined as the combination of plasma cell
proliferation disorder(typically myeloma) , polyneuropathy and effects on many other organ systems
Peak incidence around 5-6 th decade of life unlike myeloma which is common around 6-7th decade of life
Twice as common in menOther names: osteo sclerotic myeloma, pep
syndrome, crow fukase syndrome, takatsuki syndrome
DIAGNOSTIC CRITERIA
Polyneuropathy Monoclonal plasma cell disorder One or more of the following major criteria - Sclerotic bone lesion - Castleman’s disease - Elevated VEGF levels One or more of the following minor criteria Organomegaly ( enlarged spleen,liver or lymphnode) Extra vascular volume overload(pleural effusion,ascities,pedal edema)
endocrinopathy(adrenal,pitutary,gonadal,parathyroid)dm,hypothyroidism alone insufficient
Papilledema Thrombocytosis or Polycythemia Skin changes-hyperpigmentation,dermis
thickening,hirsutism,hyperhidrosis Glomerulonepritis Sensory neural hearing loss
These patients have only 5% or less than bone marrow plasma cell
Rarely have hypercalcemia or renal insufficiency
1/3rd to1/2 of pt have poly neuropathy where as in classic multiple myeloma only 1-8% of pts have polyneuropathy
EtiologyCaue is unknow
Anti body to HHV 8 seen 78% of cases with castleman disease and 22% of patients without castleman disease
Increased level VEGF, IL1BETA, IL6 and TNF alpha
VEGF targets the endothelial cells and promote angiogenesis
Both IL 1 beta and IL6 stimulate VEGF production
Elevated level of VEGF correlate with disease activity
Elevated VEGF accounts for organomegaly,edema and skin changes; but role in polyneuropathy uncertain
POLYNEUROPATHYThough resembles CIDP it is motor
predominanceMarked slowing of conduction velocity with
prolonged distal latencies and progressive dispersions of compound muscle action potentials with stimulation of motor nerves proximally
Nerve biopsy shows combination of axonal degeneration and demyelination
ORGANOMEGALY Hepatomegaly and Lymphnode enlargement
seen in 2/3rd of patientsSpleenomegaly seen in 1/3 rd of patientsBetween 11-30% patients have castleman’s
diseaseAssociation is not fully understood
MONOCLONAL PLASMA PROLIFERATIVE DISORDERMonoclonal protein is not large
May be missed on serum electrophoresis in 1/3 rd of patients
These patients require immunofixation electrophoresis of serum/urine
<10% patients have no circulating protein
They need biopsy proof of plasmocytoma
In general number of plasma cells are low and bone marrow shows hypercellular and reported as reactive or myeloproliferative disorder
SCLEROTIC BONE LESIONApproximately seen in 95% of patients
One half of patient have solitary sclerotic lesion
1/3 rd of patient have multiple sclerotic leisonPelvis ,spine, ribs and proximal extremites are
often involved
PULMONARY DYSFUNCTIONPulmonary hypertension,restrictive lung
disease and impaired diffusion capacity of carbon monoxide may occur
Improvement of PHT after therapy has been reported
Only 19% of poems syndrome initially presented with pulmonary hypertension
HAEMATOLOGY50% OF cases have thrombocytosis
20% of cases have mild erythrocytosis; anemia is not a feature of poems syndrome
May be arterial or venous thrombosis
TREATMENTCorticosteroid-15% patients shows clinical
improve ment and 7% of cases shows clinical stabilisation;should not be consider definite therapy rather temporizing therapy
Alkylating agents Cyclophosphamide-as a single agent or in
combination with prednisolone shows clinical improvement in 40% of cases
Melphalan- most effective agent against plasma cell disorder ; but there is risk of mds /acute leukemia
Thalidomide Has anti vascular endothelial growth
factor and anti tumour necrosis factor Cause peripheral neuropathy in 20%
patients receiving patients Worsen the pedal edema As a single agent no more effective
than oral alkylators
RADIATION patients with single or multiple sclerotic agent in
limited area –treated with radiation therapyWidespread sclerotic leison –systemic therapy
neededHigh dose chemotherapy and stem cell
transplantation have been triedIntravenous immunoglobulin and plasmapheresis
shows no clinical benefitBevacizumab – monoclonal antibody against vegf
have been triedAll trans retenoic acid has been used to suppress
the production of proinflammatory cytokines
PrognosisIf untreated often fatalOnly 60% of patients alive 5 yrs after onsetPrognosis is better for patient with single
sclerotic leisonsMultiple bone leison – intermediate prognosisCommon cause of death-cardiorespiratory
failure,infection and capillary leak syndrome
THANK YOUREF: HARRISON 17 EDI OTM E MEDICINE
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