A CASE OF ALLOIMMUNIZATION IN

A PATIENT OF HBE BETA

THALASSEMIA

Dr Rajesh Sonani (Presenting Author)

Dr Jaymin Bhatt

Dr Shweta Gupta

Dr Pritesh Rajani

Dr Nidhi Bhatnagar

Dr M D Gajjar

INTRODUCTION

� HbE Beta Thalassemia occurs due to

substitution of Glutamic acid by Lysine at

position 26 of the β chain.

� After HbS, it is the second most prevalent

abnormal Hemoglobin in the world, mainly in

the South-East Asian countries.

PATHOPHYSIOLOGY

� The β chain of Hemoglobin E is synthesized at a

reduced rate compared with that of Hemoglobin

A because the mutation creates an alternate

splicing site within an exon.

� This results in reduced rate of synthesis βE

chain and therefore, of HbE.

Continued..

� HbE may be present in the heterozygous

(genotype AE), homozygous ( EE or Hemoglobin

E disease) and compound heterozygous (

hemoglobin E/ β Thalassemia, Sickle

cell/hemoglobin E or SE genotype) states.

� HbE may therefore be regarded as β+

thalassemic hemoglobinopathy.

CASE REPORT

� A 23 year old female, primigravida , with history

of 7.5 months of amenorrhea was admitted to

Civil Hospital, Ahmedabad with chief complains

of abdominal pain.

PAST HISTORY

� Patient was hospitalized for anemia before 1

year where investigations showed a Positive

NESTROFT (Naked Eye Single Tube Red cell

Osmotic Fragility Test).

� Further work-up of the patient led to the

diagnosis of HbE β-Thalassemia (on High

Performance Liquid Chromatography-HPLC).

� So, at that time the patient was transfused 3

units of Packed Cell Volume at a private clinic.

Here, HbF is 53.8 % and HbA2 is 42.4% which is consistent with HbE β

Thalassemia.

HPLC of the patient

PHYSICAL EXAMINATION(At the time of admission)

� Pallor : ++

� Vitals (Temperature, Pulse, Respiratory Rate

and BP ) : within normal range

� RS : NAD

� CVS : NAD

� CNS : NAD

� Per Abdomen : Uterine size corresponding to 30

weeks of gestation with Fetal Heart Sounds

(FHS) well heard.

Laboratory findings(At the time of admission)

� Hemoglobin : 6.6 gm/dL

� Red Cell Count : 2.96 million/cmm

� Total & Differential Count : in normal reference range

� Platelet count : 1.03 lacs/cmm

� MCV : 72 fl

� MCH : 25.1 pg

� MCHC : 34.8 gm/dL

� P / S : RBCs predominantly microcytic, mild hypochromic

ULTRASONOGRAPHY FINDINGS:

� Confirmed the findings of P/A examination.

� Also revealed hepato-splenomegaly.

� Patient delivered a live, pre-term baby

vaginally weighing 1.48 Kgs after which

patient was transfused 2 units of group

compatible Packed Cell Volume (PCV) with no

adverse reaction.

� On 10th Post-partum day, patient developed

fever with Hb falling to 3.7 gm/dL for which

she was advised by treating physician to

transfuse 4 units of PCV

� But on major Cross-match, no group

compatible units were found.

SEROLOGICAL INVESTIGATIONS

� Fresh sample was obtained which was not haemolysed with no auto-clumps.

� Cell and Serum Grouping (at room temperature)

� Direct and Indirect Antiglobulin Test : Both were Grade 3 Positive

Anti

A

Anti

B

Anti

D1

Anti

D2

A Cell B Cell O

Cell

+3 0 +3 +3 0 +3 0

Antibody Screening with DiaMed 3 and 11 Cell

Panels

Cell 1 Cell 2 Cell 3

+3 0 +3

Results of 3 cell panel as follows:

Results of 11 cell panel as follows:

1 2 3 4 5 6 7 8 9 10 11

0 4+ 4+ 0 0 4+ 4+ 4+ 0 4+ 4+

RESULTS

� The above results showed the presence of Anti K

and Anti M which might have developed as a

result of allo-immunization due to previous

blood transfusions.

� The presence of Anti M antibodies was further

confirmed by using papain which turned the

previously positive result of cell 1 in 3 cell panel

negative.

MANAGEMENT

� M antigen & K Antigen negative blood was

searched using commercial Anti M & Anti K sera.

� Patient was started on steroids

(Methylprednisone) daily.

� Subsequently, the patient underwent

splenectomy during which she was transfused

same group major cross match compatible units

with no adverse reactions to transfusion noted.

� On discharge, pt’s Hb was 10.1 gm/dL and

further follow-up has shown no significant fall in

Hb.

DISCUSSION

� After development of significant antibodies,

patients receiving multiple transfusions

present challenging task in management.

� When specific antibody is identified as in this

case, that particular antigen negative blood can

be given.

� Steroids forms the first line of treatment in

these patients followed by immuno-suppresion

with azathioprine or cyclophosphamide.

� Use of IVIg and spelenectomy is indicated in

refractory cases.

THANK YOU