Download - A Case of Renal Amyloidosis
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A Case of Anasarca for Evaluation
PROF P.VIJAYARHAGAVAN’ S UNIT
C. R. RAJ KUMAR
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60 yrs male admitted with c/o generalized swelling all over the body -
20days
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H/O present illness: 20 days back pt was apparently normal
then pt C/O Swelling all over the body – 20 days -associated with abdominal distensionc/o malaise, easy fatigability + No h/o palpitation , syncope , giddinessNo h/o difficulty in breathing, PND, orthopnea No h/o abdominal pain , No h/o jaundice
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No h/o hemetemesis or melena No h/o decreased urine output,
heamaturia,or dysuria No h/o cough with expectoration No h/o hemoptysis No h/o fever No h/o altered sensorium
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PAST H/O; No h/o similar illness in the past not a known case of T2DM , SHT, BA ,TB No h/o surgery in the past FAMILY H/O; No h/o similar illness in his family
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PERSONNEL H/O ; Mixed diet Known alcoholic, smoker for past 20 yrs. He stopped alcohol for 1 ½ yrs.
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GENERAL EXAMINATION; Pt conscious, Oriented A febrile No pallor No cyanosis, No clubbing Not icteric Bilateral pitting pedal edema ++ No generalized lymphadenopathy No signs of liver cell failure
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VITALS: Pulse – 82/min BP - 110/ 80 mm/Hg JVP – normal RR -18/min
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CVS – S1S2 heard no murmurs RS - NVBS no added sounds
P/A - Distended, Free fluid +
no organomegaly CNS - NFND
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ANASARCA FOR EVALUTION
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INVESTIGATIONS; CBC; Hb -11.9 TC – 21OOO DC – P85 , L -15 ESR -5/12 PLATELETS- 1.7 lakhs PCV – 36 RBC – 3.87
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RFT: Blood sugar – 98mg Urea -28 mg Creatinine – 0.9mg
ECG: Low voltage complexes, T inversion V3 –
V6
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TOTAL COUNT 8500cells/cumm
DC POLYMORPHS 68%
LYMPHOCYTES 36%
EOSINOPHILS 6%
PCV 34.7
MCV 90.8fl
MCH 29.3pg
MCHC 32.3g
HB 11.2gm
ESR 38mm/ hr
PROTHROMBIN TIME TEST -14
APTT 26
PLATELETS 79000
RBC 3.8 million
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RFT
SUGAR 90mg
UREA 18mg
CREATININE 0.7mg
LFT
TOTAL BILIRUBIN 0.42mg
DIRECT 0.28mg
AST 33u
ALT 17
ALK PHOSPATASE 159
GGT 23
TOTAL PROTEIN 4mg
ALBUMIN 1.2mg
GLOBULINS 2.8mg
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ELECTROLYTES
SODIUM 131.3meq
POTTOSIUM 4.33meq
CHLORIDE 94.3meq
SEROLOGY
HIV NEGATIVE
ANTIHCV NEGATIVE
HbsAg POSITIVE
URINE
SUGAR NIL
ALBUMIN +
DEPOSITS 4-6 pus cells, no RBC,no casts
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LIPID PROFILE: Serum total cholesterol – 204mg Total triglyceride - 230 mg LDL- 110 VLDL- 180 HDL - 40
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USG ABDOMEN: Normal sized kidneys with mild increase in
cortical echogenicity Minimal ascities + Normal liver echo texture GB, CBD, Portal vein, pancreas, spleen,
bladder and prostate
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Pt was treated with symtomatically with diuretics Inj frusemide and conservative management
Meanwhile 24 hrs urinary protein was send Blood culture , urine culture was send
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Meanwhile pt c/o decreased urine output -increasing swelling all over the body with facial
puffiness Repeat RFT was taken It shows increased renal parameters 1.9mg
2.9mg
3.8mg
SUSPECTED : AKI – Mutifactorial- diuretics (?pre-renal/?cast nephropathy)/
sepsis
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Nephrology opinion obtained ?Nephrotic syndrome
Suggested -Viral markers
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24 hrs urinary protein: -1250mg /day
Urine Bence Jones protein- negative
Serum calcium - 9.2mg
Thyroid function test – normal
Peripheral smear study - normal
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CARDIAC EVALUATION: concentric LVH
No RWMANormal LV systolic functionMR mild
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SERUM IMMUNO ELECTROPHORESIS
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Serum immuno electrophoresis: IgA - 447.1mgmg/dl [70 – 400mg/dl] IgM - 381.55mg/dl [700 – 1600mg/ml] IgG - 67.25mg/dl [40-230mg/dl] IMPRESSSION: IgA monoclonal gammapathy
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Bone marrow:normal study
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RENAL BIOPSY
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Renal Biopsy (Microscopic description): -blood vessels are thickened and show
minimal a cellular material which are CONGO RED positive
-There are multiple foci of tubular atrophy. apple green bifringence seen under polarized light which is resistant to pre treatment by potassium permanganate
-Immmunoflurecence stain shows minimal mesangial deposits of IgM
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FINAL REPORT OF RENAL BIOPSY:
Renal biopsy showing features of AMYLOIDOSIS [NON –AA]
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After 10 days pt RFT was normal after stopping diuretics
and coverage with broad spectrum antibiotics
-serum creatinine - 0.8mg -24 hrs urinary protein – 950 mg -pt symptomatically improved
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FINAL DIAGNOSIS: IgA monoclonal gammapathy Primary amyloidosis AL [non AA] AKI recovered HbsAg positive (possibly explaining IgM
deposition in renal biopsy)
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Journal of TransplantationVolume 2009 (2009), Article ID 103784, 3 pagesdoi:10.1155/2009/103784Case Report
Hepatitis B Associated Monoclonal Gammopathy That Resolved after Successful Liver Transplant
P. Sreenivasan and S. Nair
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The role of hepatitis B surface antigen in Nigerian children with nephrotic syndrome.
Abdurrahman MB, Fakunle YM, Whittle HC. Abstract Hepatitis B surface antigen was detected by
radioimmunoassay in the sera of 18 out of 50 (36%) children with nephrotic syndrome and in 28 of 61 (45.9%) controls. Immunofluorescent studies of kidney biopsies showed HBsAg, IgG, IgM and C3 deposits in a granular pattern in the biopsies of 12 children with nephrotic syndrome and in none of the control kidney biopsies, even though there was no significant difference between the frequencies of HBsAg in the sera of these two groups. We conclude that these findings are indicative of an aetiologic role for HBsAg in these patients
PMID: 6191643 [PubMed - indexed for MEDLINE] Publication Types, MeSH Terms, Substances
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Monoclonal gammopathy of undetermined significance (MGUS) has been most commonly associated with diseases like
-multiple myeloma, -Waldenstrom's macroglobulinemia, -primary systemic amyloidosis, and other lymphoproliferative disorders. There has been an isolated report of MGUS in
patients coinfected with HIV and Hepatitis B, as the work by Amara et al. in 2006. Here, we report a case of IgA-kappa light chain gammopathy secondary to Hepatitis B infection, which resolved after liver transplantation.
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MONOCLONAL GAMMOPATHY
M protein in the serum without symptoms or signs of multiple myeloma, macroglobulinemia, amyloidosis, or lymphoma. Less than 10% plasma cells in the bone marrow.
The incidence of monoclonal gammopathy of uncertain significance (Monoclonal Gammopathy of Uncertain Significance ) increases with age and may approach 3% in persons 70 years of age or older
Lymphoid malignancies, amyloidosis, or multiple myeloma will develop in as many as one-third of patients with apparently benign monoclonal gammopathies.
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Two major types of MGUS :-IgM MGUS and-non- IgM MGUS [mostly
comprised of IgG and IgA]
Patients with non-IgM MGUS progress to multiple myeloma at a rate of 1% per year
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No specific therapy is necessary, but close observation is required.
MGUS patients should be periodically monitored for changes in serum M proteins, urinary Bence-Jones proteins, evidence of renal failure, anemia, hypercalcemia, lytic bone lesions, or bone marrow plasmacytoses.
Risk of developing a malignant disorder is 12% at 10 years, 25% at 20 years, and 30% at 25 years.
favorable prognosis concentrations of homogeneous immunoglobulin less
than 2 g/dL, no increase in concentration of the immunoglobulin from
the time of diagnosis, no decrease in the concentration of normal
immunoglobulins, absence of a homogeneous light chain in the urine, and normal hematocrit and serum albumin
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Disease M-protein type Associated clinical features
Monoclonal Gammopathy of Uncertain Significance (MGUS)
Any in small quantity
None
Multiple Myeloma IgG, IgA, light chain only, IgD
CRAB*
Lymphoplasmacytoid-Cell Lymphoma (Waldenström's Macroglobulinemia)
IgM OVA*
amyloidosis Any of the above
Protein deposition in multiple organ systems
*see below
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Underlying disorder Effects
MGUS, MM, WM Light chains metabolized to form amyloid which accumulates in organs
Organ infiltrated Effects
Heart Diastolic dysfunction - CHFLiver/spleen Enlargement
Kidney Nephrotic syndrome, renal failure
Nerves Neuropathy
Skin Bleeding, bruising
Gut Large tongue. Dysmotility
Treatment
Chemotherapy
Prognosi
Poor: average survival about 1 year (Kyle 1997)
AL AMYLOIDOSIS•Definition•Clinical manifestations (examples only – there are lots more)
Treatment Chemotherapy
Prognosis Poor: average survival about 1 year (Kyle 1997)
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