¢ Raashid Ali, a 12 year old boy, resident of Gujjar Khan was brought by his parents on 7th Jan, 09 with:
¢ P/C:
- Anuria I
- Vomiting I 2 days
- Bilateral Flank pain I
¢ Past Hx:
Swollen face 1 month back which was treated
Fever & diarrhea in the past week (after eating
‘Pakorray’)
¢ Family Hx:
No H/O Diabetes mellitus or any malignancy
¢ Treatment Hx:
No H/O recent surgery or burns
Examination:
A young child well-oriented in time, place & person is lying comfortably in bed with:
¢ Pulse: 80/min
¢ Temp.: 98O F
¢ B.P.: 120/80 mm Hg
¢ Pallor: ++
¢ Jaundice/Edema?: absent
¢ Skin: normal
¢ Lymph nodes: not palpable
¢ G.I.T.: mild tenderness in flanks
¢ C.N.S./Resp./C.V.S.: unremarkable
Provisional diagnosis
¢ Acute renal failure ß ?
¢ Inj. Furosemide
¢ Inj. Calcium gluconate
¢ Inj. NaHCO3
¢ Inj. Ceftriaxone Na
¢ Inj. Venofer if Hb <10 g/dL
¢ 2 pints blood to be arranged
¢ He passed urine the very day of Rx!
Lab. Investigations
¢ TLC: 24000/micro.L (A.N.C.=21000/micro.L)
¢ RBC: 2.3 (109 )
¢ Hb: 5 g/dL
¢ MCV: 75 fL
¢ MCH: 24 pg
¢ RDW-SD: 39, RDW-CV: 15
¢ PLT: 87 000/uL
¢ Peripheral Blood Morphology:
Rouleaux formation+++
Neutrophilia & left shift
Bicytopenia
RBC fragmentation+
¢ No M.P. seen
¢ Retics: 4.5%
¢ Prothrombin Time:
Patient: 22 sec
Control: 14 sec
¢ Urine R/E:
Albumin+
RBCs Numerous
No crystals/sugar
¢ 24 hr urinary protein: 2.8 g (<0.15 g)
¢ USG abdomen:
Renal parenchymal disease,
Kidney size normal
¢ ANA [AIHA]: Negative
¢ Anti-MPO (p-ANCA) [Vasculitides]: Negative
¢ Anti-proteinase 3 (c-ANCA) [WG] : Negative
¢ Viral serology for Hepatitis B, C: Negative
Chemistry:
¢ Creatinine: 173 micro.mol/L (60 – 156 micro.mol/L)
¢ Urea: 20 mmol/L (3.3 – 8.3 mmol/L)
¢ S. Potassium: 8.4 mmol/L (3.4 – 5 mmol/L)
¢ LDH: 1250 U/L (225 – 450 U/L)
¢ Bilirubin: 9 micro.mol/L (<17 micro.mol/L)
[Indirect: 4 micro.mol/L (< 12 micro.mol/L]
¢ ALT: 19 U/L (<41U/L)
¢ S. Albumin: 18 g/L (35 – 50 g/L)
¢ S. Cholesterol: 3.6 mmol/L (5.7 mmol/L)
¢ S. Triglycerides: 5.6 mmol/L (<1.8 mmol/L)
¢ IMPRESSION:
¢ LEUKOCYTOSIS
¢ THROMBOCYTOPENIA
¢ MICROANGIOPATHIC HEMOLYTIC ANEMIA
Differential Diagnosis5:
¢ H.U.S.
¢ T.T.P.
¢ D.I.C.
¢ Renal parenchymal diseases
¢ I.T.P. (Evan’s syndrome)
Final Diagnosis:
¢ Haemolytic-Uremic Syndrome
Rx:
¢ Corticosteroids (Inj. Hydrocortisone 100 mg TDS)
¢ 4 units R.C.C transfused
¢ Raashid Ali improved and was discharged with a Creatinine level of 90 micro.mol/L
(60 – 156 micro.mol/L).
Haemolytic –Uremic Syndrome
Microangiopathic haemolytic anaemia, renal failure, and thrombocytopenia without the features of D.I.C.
¢ Diarrhea-related:
Exotoxin of E. coli/ S. dysenteriae
¢ Familial:
Factor H deficiency (Complement regulatory protein)
¢ Atypical:
Post-partum
Pathogenesis
¢ Trigger à damages endothelium à endothelial injury & activation à High Endothelin4, vWF multimers8 & Low PGI2 secretion à vasoconstriction & platelet aggregation4
¢ Verocytotoxin of E. coli can directly activate platelets4
¢ Consumptive thrombocytopenia6
¢ Ischaemic damage6 of renal cortex1
- Viral infections
-
Drugs7:
- quinine, , ticlopidine, clopidogrel,
cyclosporine, &
mitomycin C
-
Radiation therapy
-
Bone marrow transplantation
-
Factor H deficiency4
¢
High
LDH & Indirect bilirubin
¢
Coagulation
abnormalities in HUS are milder from those observed in classic DIC:
the PT and aPTT are normal or only slightly prolonged.8
¢
No
deficiency of vWF-cleaving metalloprotease (ADAMTS13)
¢
Low
serum C3 level in Factor H deficiency
Drug
treatment:
¢
Diarrhea-related
HUS
¢
In
children, self-limited. Conservative Rx of Renal failure .1, 5
¢
Antimotility
drugs are contraindicated.2
¢
Antibiotics:
Sepsis or S. dysenteriae.2
¢
Corticosteroids
can be beneficial.9
¢
Dialysis
in resistant cases.9
¢
Familial
& Atypical HUS
¢
In
adults, large-volume plasmapheresis with FFP2 (upto 80mL/kg) daily
until in remission.5
Take-home
message
References:
Books:
1:
William’s Haematology, 7th Edition
2:
Postgraduate Hoffbrand, 5th Edition
3:
Oxford Handbook of Hematology
4:
Pathologic Basis of Disease, 7th Edition
5:
Current Medical Diagnosis & Treatment, 2009 Edition
Journals:
6:
Haematologica
7:
Current Opinion in Haematology
8:
The New England Journal of Medicine
Articles: