Haematology Watch

¢  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


- E. coli O157: H7 gastroenteritis, S. dysenteriae

- 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

  1. Undercooked food should be avoided.
  2. Diarrhea in children should be investigated for aetiology.
  3. Preventable re-exposures (e.g. drugs) should be avoided.
  4. Antimotility agents should be avoided in infectious diarrhea.
  5. HUS should be in D/D of Renal failure in children.

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: