1. Case presentation Immune Hemolytic Anemia
In the name of God
Case presentation
Immune Hemolytic Anemia

2. Case presentation Patient:Female DOB : 9/10/95 ( 29/12/2016) Age: 5MO
Second child , parents: First degree, 37 weeks, HC: 36cm, gr, C/S duo to decrease of fetal movement
First Child : OK
Admission :Amir kola Hospital :9/10/95
CC & PE: Dyspnea , Cyanosis, Hepatosplenomegaly, Edema ,Decrease O2 saturation (So2 < 30%)
VBG: PH: PCO2: 79 SO2 : 41% HCO3 : 22

3. Work up Impression : CHD and PFC
Diagnosis:Hydrops Fetalis - Non immune
First CBC:
WBC: RBC: Hb: 4.5
MCV: MCH : MCHC: Plate : 85000
Retic : 1.4 % D-Coombs: neg
SGOT : SG PT : Alb: BilT/D:2.3 / 0.3
G6PD : sufficient CRP : 14 high (135 , 68)
BG neonate : O Mother A+
CXR:Cardiomegaly
ECHO: RVE , LVE, TR, MR
Abdo Sonography : large liver , spleen Nl 11/10/95

4. Work up Anti ParvoVirus : Neg 22/10 /95 (PCR PVB19 :???)
Torch study & metabolic disorders
Management:
Intubation , Ventilation, AB-therapy , Surfactant & PG ,Milrinon, Isovolume Exchange 165 cc ( 9/10/95 ) , packed cell transfusion ( 10/10 45cc + lasix)
Discharge ; 26/10/95 good condition

5. Follow Up in AMIR KOLA HOSPITAL
Follow Up : mo old ; visit : Anemia
Recommendation EP ,( Hb :7) At first good response ,( Hb 10)
Again anemia :
7/2/96 Admisson & visit of Hematologist
WBC: RBc: Hb: MCV : 86 MCh: 29 MCHC: 33
Plate : Nl Retic : 0.5 % Coombs : neg
7/2/96 PC transfusion
Mother;RBC : Hb : MCV : MCH : 23
HbE: Nl A2 2.5 % A; 97.5
Father: RBC: Hb: 14 MCV : 88 NL Hb : NL
Erythropoietin level:116 high(9/2/96)
Hb E patient : HB Barts: 43%??? HBA:50% HbA2:1.9% HbF;4.25% (13/2/96)

6. Follow Up in AMIR KOLA HOSPITAL
16/2/96 : HB: Coombs : 1+ pos
PB19 Mother : Neg
Infant PV B19 IgG; IgM: 2.3pos (11/2/96) IVIG: Infusion
23/2/96 :Hb : 6

7. Work up / Mofid Hospital
Admission in Mofid hospital 25/2/ Age :4. 5 mo
PE: good baby , mild icter in sclera, Hepatomehgaly , pallor
WBC : N ; 51 % L ; 43
RBC: 1.800, Hb : 5
MCV: MCH : 27 Plate : RDW: Retic ; <0.1 %
Coombs : Direct : Indirect : 1+
Aniso, Hypo , Poikilo ,Sper
ALK : 986 Nl SGOT ; SGPT : LDH : 1156
Urine ; nl
Coombs D/I ,Ab scrennng mother: Neg
Antibody screning patient ; Tube method : Auto control: Pos 4+
Strong Warm AutoAb IgG; Complement C3D : positive : 1+
Drugs : IVIG 1gr/kg x 4 days & Cortico (CS)

8. Work up / Mofid Hospital
CXR
ECHO :Nl
Abd Sono: Liver : 75mm,liver span increase Spleen : Nl size
BMA : Increased Cellularity in M & E series 28/2/96

9. Bone Marrow

10. Bone Marrow

11. Bone Marrow

12. Work up / Mofid Hospital
Virology :Toxo (Patient) : IgG , IgM +( mild incease)
Infectous consult : No treatment
Eye Exam: Nl
Brain CT : Benign subarachnoid enlargment, No calcification
EBV & CMV: Neg PCR
Parvovirus B19 PCR: positive
IG: Nl - IgG increased ( IVIG infusion)
Collagen vascular tests patient & Mother : Negative
Mother Torch : Neg
Thalassemia Gene Analysis: final result-Pending

13. Follow up / Mofid hospital
Duo to progressive rising of Retic to 14-15% & high level of LFT and Gamma GT: 193
GI consult : Immune , Viral , Giant cell hepatitis
Biopsy of Liver was done
Puls MP and 1 gr/kg IvIg and Azathioperine 2mg/kg /day/po
Duo to decrease Hb to 6.5 and , persistant high Retic;Rituxmab :375mg/m2 / Iv infusion x 4weeks

14. Mofid hospital/ Lab tests
14/3
11/3
9/3
7/3
6/3
3/3
2/3
29/2
28/2
26/2
Date
8.6 9
9.6
9.9 11
6.9
7.8
11.4
6.5 10 5 Hb 93 87 90 84 81 79 78 73
MCV
10%
15%
13% -
11 %
14% 8% 7%
<0.1
<0.5
Retic
104
131
235
103
156
488
340
SGOT
245
253
545
262
378
271
546
SGPT
2.8/
1.2
0.5
5.5/
0.7
3.2/
1.8
3.7/
0.6
2.7/1.3
BilT/D
900
1800
1151
LDH

15. Recent Lab tests Last CBC :20/3/96 after Rituximab 2 RBC : 2.460.000
Hb: MCV: MCH MCHC: 36
Retic : 5.5% Coombs: 2+
SGOT : SGPT:188
BILT/D: 2.2/0.9
Drugs: Azathioperinr and prednison( On taper), Acid Folic
pathology Report of liver Biopsy:
Mild Giant Cell transformation ,Cholestasis, Mild microvesicular steatosis, and some asinar formation, work up for Metabolic disese

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22. Thank You