A Case of Anasarca for Evaluation

PROF P.VIJAYARHAGAVAN’ S UNIT

C. R. RAJ KUMAR

60 yrs male admitted with c/o generalized swelling all over the body -

20days

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

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

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

PERSONNEL H/O ; Mixed diet Known alcoholic, smoker for past 20 yrs. He stopped alcohol for 1 ½ yrs.

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

VITALS: Pulse – 82/min BP - 110/ 80 mm/Hg JVP – normal RR -18/min

CVS – S1S2 heard no murmurs RS - NVBS no added sounds

P/A - Distended, Free fluid +

no organomegaly CNS - NFND

ANASARCA FOR EVALUTION

INVESTIGATIONS; CBC; Hb -11.9 TC – 21OOO DC – P85 , L -15 ESR -5/12 PLATELETS- 1.7 lakhs PCV – 36 RBC – 3.87

RFT: Blood sugar – 98mg Urea -28 mg Creatinine – 0.9mg

ECG: Low voltage complexes, T inversion V3 –

V6

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

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

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

LIPID PROFILE: Serum total cholesterol – 204mg Total triglyceride - 230 mg LDL- 110 VLDL- 180 HDL - 40

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

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

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

Nephrology opinion obtained ?Nephrotic syndrome

Suggested -Viral markers

24 hrs urinary protein: -1250mg /day

Urine Bence Jones protein- negative

Serum calcium - 9.2mg

Thyroid function test – normal

Peripheral smear study - normal

CARDIAC EVALUATION: concentric LVH

No RWMANormal LV systolic functionMR mild

SERUM IMMUNO ELECTROPHORESIS

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

Bone marrow:normal study

RENAL BIOPSY

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

FINAL REPORT OF RENAL BIOPSY:

Renal biopsy showing features of AMYLOIDOSIS [NON –AA]

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

FINAL DIAGNOSIS: IgA monoclonal gammapathy Primary amyloidosis AL [non AA] AKI recovered HbsAg positive (possibly explaining IgM

deposition in renal biopsy)

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

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

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.

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.

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

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

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

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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