Case presentation Immune Hemolytic Anemia In the name of God Case presentation Immune Hemolytic Anemia
Case presentation Patient:Female DOB : 9/10/95 ( 29/12/2016) Age: 5MO Second child , parents: First degree, 37 weeks, HC: 36cm, 4400 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: 7.05 PCO2: 79 SO2 : 41% HCO3 : 22
Work up Impression : CHD and PFC Diagnosis:Hydrops Fetalis - Non immune First CBC: WBC: 22300 RBC: 1.33 Hb: 4.5 MCV: 112 MCH : 33 MCHC: 30 Plate : 85000 Retic : 1.4 % D-Coombs: neg SGOT : 38 SG PT : 28 Alb: 2 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
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
Follow Up in AMIR KOLA HOSPITAL Follow Up : 1. 5 mo old ; visit : Anemia Recommendation EP ,( Hb :7) At first good response ,( Hb 10) Again anemia : 7/2/96 Admisson & visit of Hematologist WBC: 13000 RBc: 2.06 0.000 Hb: 6 MCV : 86 MCh: 29 MCHC: 33 Plate : Nl Retic : 0.5 % Coombs : neg 7/2/96 PC transfusion Mother;RBC :5.800000 Hb :13.7 MCV : 71 MCH : 23 HbE: Nl A2 2.5 % A; 97.5 Father: RBC: 4.45 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)
Follow Up in AMIR KOLA HOSPITAL 16/2/96 : HB: 6 Coombs : 1+ pos PB19 Mother : Neg Infant PV B19 IgG;0.5 IgM: 2.3pos (11/2/96) IVIG: Infusion 23/2/96 :Hb : 6
Work up / Mofid Hospital Admission in Mofid hospital 25/2/96 Age :4. 5 mo PE: good baby , mild icter in sclera, Hepatomehgaly , pallor WBC : 23300 N ; 51 % L ; 43 RBC: 1.800,000 Hb : 5 MCV: 73 MCH : 27 Plate : 540000 RDW:17 Retic ; <0.1 % Coombs : Direct : 4+ Indirect : 1+ Aniso, Hypo , Poikilo ,Sper 1-2 + ALK : 986 Nl SGOT ; 340 SGPT : 546 LDH : 1156 Urine ; nl Coombs D/I ,Ab scrennng mother: Neg Antibody screning patient ; Tube method : Auto control: Pos 4+ Strong Warm AutoAb IgG; 3+ Complement C3D : positive : 1+ Drugs : IVIG 1gr/kg x 4 days & Cortico (CS)
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
Bone Marrow
Bone Marrow
Bone Marrow
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
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
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
Recent Lab tests Last CBC :20/3/96 after Rituximab 2 RBC : 2.460.000 Hb: 8.6 MCV: 95 MCH 34 MCHC: 36 Retic : 5.5% Coombs: 2+ SGOT :170 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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