Case presentation prof. gamal a. tawfik

1. Prof. DR.Gamal A. Tawfik
Professor of Internal Medicine and Nephrology
DRBasma Osman Ibrahim
Assist.ant Lecturer of Internal Medicine &
Nephrology
Suez Canal University

2.  A.M.A is a 42 year old male, worker
lives in Suez, married and has 2 children.
 He presented with bilateral marked lower
limb edema with abdominal swelling.
 He is heavy cigarette smoker, addict to
tramadol and cannabis

3.  The patient was leading apparently healthy life till
2 monthes ago when he noticed bilateral lower
limb edema of gradual onset, progressive course ,
pitting in nature, involving both lower limbs and
abdominal wall with abdominal swelling and
infrequent attacks of epistaxis and fresh bleeding
per rectum(FBPR).

4. Local Examination:Vitally :Generally :
•Heart Examination :
NAD
•BP: 110/70 mmHg•Looks ill , cachectic
orthopenic,
conscious oriented to
time place and
persons.
•Bilateral lower
limb pitting edema
extending to involve
all the limb and also
abdominal wall and
sacral edema
•Finger clubbing
with leukonychia.
•Chest examination :
Bilateral basal
diminished
intensity of breath
sounds.
•Respiratory rate:
25 cycle/min.
•Temperature: 37°c
(axillary)
•Abdominal
Examination :
Moderate ascites.
•Pulse: 88 beat/min.,
regular, equal on both
sides of average volume
with intact peripheral
pulsations.
•On presentation :

5. WITH THE FOLLOWING INVESTIGATIONS DONE ON
ADMISSION:
PLT →350 x 103/mm3TLC→ 11 x 103 /mm3Hb → 15 gm/dl
S.K→ 3 mmol/LS.Na→ 129 mmol/Ls.cr→1 mg/dl
mg/dl3.5S.Uric acid→S.Calcium → 7.3mg/dlS.Albumin → 1.7gm/dl
s.bilirubin (T)→0.3 mg/dl
(D) →0.08 mg/dl
ALT → 21U/L
U/L41AST →
Total Protein→ 3.5
gm/dl
TSH → 4.2mIU/mL
T3 → 27ng/dL (↓↓)
T4 → 34 nmol/L(↓↓)
LDH → 305IU/LESR→ >100
Albumin/creatinine
ratio→ 2 gm/mmol
CRP→ 3.5 mg/dl
S.Cholesterol → 605mg/dl
S.Triglyceride →385mg/dl
LDL → 491mg/dl
HDL → 81mg/dl
24 hour urine collection
for protein was 8 gm/d
Urine analysis: PTN +,
pus cells( 5-10/HPF),
RBCs (5-10/HPF)

6. o ECG : NAD
o CXR: bilateral obliteration of costopherenic angels
with increased bronchovascular markings.
o Pelvi-abdominal US :
 Mild- moderate intra-peritoneal free fluid
collection
 Liver and spleen : NAD
 Kidneys : LK 11 cm, RK 10.5 cm with
echogenicity grade II bilaterally, no masses,
backpressure changes or stones.

7.  Patient started to complain of generalized
abdominal pain with no relieving or aggravating
factors and was associated with deterioration of
his kidney function as serum creatinine showed
gradual elevation with the following findings on
examination :
 as blood pressure dropped to 80/50 - 90/60 mmHg
 Ascites became marked and causing distress to
patient that direct us to do paracentesis of 5 liters
with albumin infusion and samples of tapped fluid
were sent for chemical , cytological and culture &
sensitivity.

8.  Chemical analysis of ascitic fluid revealed TLC
400/ mm3 - LDH 56 IU/L - glucose 128mg/dl
 Serum creatinine reached 2.5 mg/dl - TLC
reached 20X 103 /mm3(61% neutrophils )
 Patient was kept on:
 Lasix 40 mg twice daily
 Cefobid 1gm IV twice daily
 Ator 20 mg once daily

9.  Ascites was rapidly accumulating
inspite of frequent tapping of small
volumes(4 litters each time ) with
inadequate response to diuretics and
more deterioration of S.Cr, also liver
became tender and palpable 4
fingers below costal margin.

10.  Follow Up US was done showing : enlarged liver
18.5 cm and patent portal vein , spleenomegally 13.5
cm , Rt kidney 15 cm and Lt kidney 14 cm with
patent renal vessels.
 Echocardiography : mild localized pericardial
effusion related to Rt atrium with no evidence of
tamponading
 Serum creatinine reached 5mg/dl with metabolic
acidosis and generalized anasarca so we started
hemodialysis with ultrafiltration to facilitate further
investigation.

13.  Renal Biopsy was done.
 Serum creatinine reached 6 , TLC became
21X103 /mm3and Alb. /cr. ratio 5.3 and HD
sessions were continued.
 We decided to start solumedrol mini-pulses
(250mg/day ) for 4 days with antimicrobial
coverage of Septrin and maxipem till report
of biopsy .

14. Renal biopsy :
 46 glomeruli , out of which 2 were
obsolescent.

15. The glomeruli showed mild mesangial matrix expansion
by homogenous pink deposits having weak PAS
reactivity.

16. Tubules showed injury within proximal tubules by
intracytoplasmic crystals& droplets.

17. TUBULAR CRYSTALS MASSON TRICHROME

18. TUBULAR CRYSTALS MASSON TRICHROME

19. •
Congo red: amyloid deposits within glomeruli,
interstitium and arterioles.

20. • Congo Red Under Polarized Microscope:
amyloid deposits within glomeruli, interstitium and
arterioles .

21. Immunohistochemistry :
lambda +ve amyloid deposits & intratubular
crystals

22. Immunohistochemistry :
lambda +ve amyloid deposits & intratubular crystals

23. kappa negative

24.  Conclusion :
o Light microscopic & immunoperoxidase
findings are compatible with :
 Renal amyloidosis ,light chain type (AL-
Lambda)
 Light chain proximal tubulopathy (Lambda)
o Additional features : mild interstitial fibrosis
& tubular atrophy 20% , moderate
arteriosclerosis & mild arteriolosclerosis.

25.  Laboratory results that were pending :
23X103/mm3
(61% neutrophils,
29% lymphocytes,
9% monocytes)
•TLC
-ve
•CEA ,
•CA19-9 ,
• PSA ,
•HBsAg
mg/dl5.1•CRP+VE•HCVab
-ve•ANA65, 16 mg/dl
(Normal)
•C3, c4
Hypo-gammaglobulinemia Kappa light
chain
•Protein
electrophoresis

26.  After ending of mini-solumedrol pulses , he
was kept on dexamethazone 2 ampules / day
for 8 days. Also heparin was added for 3 days
and replaced with Aspocid due to recurrence
of FBPR & epistaxis .
 Serum creatinine continued elevation till
became 9mg/dl.
 Bone marrow aspiration done showing :
Normal Bone Marrow Picture with frequent
late erythroid forms.

27.  Patient received melphalan ( Alkaran) 2
mg 4 tablets /day for 4 days according to
oncology recommendations and he was
discharged for follow up and regular
hemodialysis .