Delayed hemolytic transfusion reaction presenting as a painful crisis in a patient with sickle cell anemia

38

Antonio Fabron Junior Gilberto Moreira Junior José Orlando Bordin

Case R eport

REVISTA PA ULISTA DE M EDIC IN A

Medical Journal

INTRODUCTION

Red blo o d cell (RBC) transfusio ns are widely used in the manag ement o f patients with sickle cell anemia (SCA). W e repo rted recently that the c a lc ula te d risk o f RBC a llo immuniz a tio n pe r transfused unit in Brazilian patients with SCA is lo wer than that repo rted by studies fro m N o rth America (1 .1 5 vs. 3 .1 0 %), reflecting a mo re co nco rdant RBC pheno type between blo o d do no rs and recipients in Brazil.1 ,2 Therefo re, we have no t b e e n ro utine ly p e rfo rming e xte nd e d RBC pheno typing o n all SCA patients prio r to initiating a transfusio n pro gram. Nevertheless, in the present repo rt we describe the o ccurrence o f a delayed hemo lytic transfusio n reactio n (DHTR) presenting as a painful crisis in a patient with SCA in Brazil.

De laye d he molytic transfusion re action pre se nting

as a painful crisis in a patie nt with sickle ce ll ane mia

Universidade Federal de São Paulo/Escola Paulista de Medicina, São Paulo, Brazil

Figure 1 - Delayed hemolytic transfusion reaction presenting as a sickle cell crisis in a Brazilian patient with sickle cell anemia. The arrows indicate the packed RBC units transfused.

ABSTRACT

Contex t: Patients with sickle cell anemia (SCA) are frequently transfused with red blo o d cells (RBC). Recently, we repo rted that the calculated risk o f RBC

allo immuniz atio n per transfused unit in Braz ilian patients with SCA is 1 .1 5 %. W e describe a delayed hemo lytic transfusio n reactio n (DHTR) presenting as a painful crisis in a patient with SCA.

Ca se report: A 3 5 -year-o ld Brazilian female with ho mo zyg o us SCA was admitted fo r a pro g ram o f partial exchang e transfusio n prio r to cho lecystecto my. Her blo o d g ro up was O RhD po sitive and no atypical RBC allo antibo dy was detected using the indirect antig lo bulin technique. Pre-transfusio nal hemo g lo bin (Hb) was 8 .7 g / dL and iso vo lumic partial exchang e transfusio n was perfo rmed using 4 units o f ABO co mpatible packed RBC. Five days after the last transfusio n she develo ped g eneralized jo int pain and fever o f 3 9 °C. Her Hb level dro pped fro m 1 2 .0 g / dL to 9 .3 g / dL and the unco njug ated bilirrubin level ro se to 2 7 mmo l/ L. She was jaundiced and had hemo g lo binuria. Hemo g lo bin electro pho resis sho wed 4 8 .7 % HbS, 4 6 .6 % HbA1, 2 .7 %

HbA2, and 2 .0 % HbF. The patient’s extended RBC

pheno type was CDe, K-k+, Kp(a-b+), Fy(a-b-), M+N +s+, Le(a+b-), Di(a-). An RBC allo antibo dy with specificity to the Rh system (anti-c, titer 1 :1 6 .3 8 4 ) was identified by the indirect antig lo bulin test. The Rh pheno type o f the RBC used in the last packed RBC transfusio n was CcDEe. The patient was discharg ed, asympto matic, 7 days after admissio n.

Key W ords: Sickle cell anemia. Transfusio n reactio n. Painful crisis.

39

CASE REPORT

A 3 5 -ye a r-o ld Bra z ilia n fe ma le w ith ho mo zygo us SCA was admitted fo r a pro gram o f p a rtia l e xc ha ng e tra nsfusio n p rio r to cho lecystecto my. Her blo o d g ro up was O RhD po sitive and no atypical RBC allo antibo dy was detected using the indirect antiglo bulin technique. Pre-transfusio nal hemo glo bin (Hb) was 8 .7 g/ dL and iso vo lumic partial exchange transfusio n was performed using 4 units of ABO compatible packed RBC. Figure 1 sho ws the serial Hb levels in relatio n to the blo o d transfusio n pro gram. Five days after the last transfusio n she develo ped generalized jo int pain and fever o f 3 9 °C. Her Hb level dro pped fro m 1 2 .0 g/ dL to 9 .3 g/ dL and the unco njugated b ilirrub in le ve l ro se to 2 7 mmo l/ L. She wa s jaundiced and had hemo glo binuria. Hemo glo bin electro pho resis sho wed 4 8 .7 % HbS, 4 6 .6 % HbA1,

2 .7 % HbA2, and 2 .0 % HbF.

The patient’s extended RBC pheno type was CDe, K-k+, Kp(a-b+), Fy(a-b-), M+N +s+, Le(a+b), Di(a-). An RBC allo antibo dy with specificity to the Rh system (anti-c, titer 1 :1 6 .3 8 4 ) was identified by the indirect antig lo bulin test. The Rh pheno type o f the RBC used in the last packed RBC transfusio n w a s C c DEe . The p a tie nt w a s d isc ha rg e d , asympto matic, 7 days after admissio n.

DISCUSSION

It has been reported that the prevalence of DHTR in patients with SCA ranges fro m 0 .7 to 4 %.2 ,3 Moreover, there are few published reports of cases in which the development of DTHR presents as a painful crisis.2,4 Although the RBC alloimmunization rate among multitransfused Brazilian patients with SCA is lower than previously reported rates in the United States,1,2 the present case illustrates that DHTR occurs and can easily be mistaken for a painful sickle cell crisis, even in co untries with lo w antig en mismatching between blo o d do no rs and SCA

recipients. Interestingly, in contrast to the fact that about 40% of the potentially hemolytic RBC alloantibodies are usually not detected even after long-term follow-up, the presence o f the abo ve-mentio ned RBC alloantibody (anti-c, titer 1:16) is still demonstrable in o ur patient’s serum 5 years after her last blo o d transfusion. In conclusion, this case reveals some of the problems associated with the prevention and diagnosis of DHTR in patients with SCA transfused in countries where the rates of phenotypic incompatibility between donors and recipients are low.

REFERENCES

1. Mo re ira Jr G, Bo rd in JO, Kuro d a A, Ke rb auy J. Re d b lo o d c e ll allo immunizatio n in sickle cell disease: The influence o f racial and antigenic pattern differences between do no rs and recipients in Bra-zil. Am J Hemato l 1996;52:197-200.

2. Co x JV, Steane E, Cunningham G, Frenkel EP. Risk o f allo immunizatio n and delayed hemo lytic transfusio n reactio ns in patients with sickle cell disease. Arch Intern Med 1988;148:2485-9.

3. Vichinsky EP, Earles A, Jo hnso n RA, Ho ag MS, Willians A, Lubin B. Allo immunizatio n in sickle cell anemia and transfusio n o f racially unmatched blo o d. N Engl J Med 1990;322:1617-21.

4. Cummins D, Webb G, Shah N, Davies SC. Delayed haemo lytic trans-fusio n reactio ns in patients with sickle cell disease. Po stgrad Med 1991;67:689-91.

Fro m Disciplina de Hemato lo g ia e Hemo terapia da Universidade Federal de São Paulo / EPM, São Paulo , Brazil

Authors

Antonio Fa bron Junior - Do cente da disciplina de Hemato lo g ia e Hemo terapia da faculdade de Medicina de Marília e pó s-g raduando da UN IFESP/ EPM

Gilberto M oreira Junior - Médico da disciplina de Hemato lo g ia e Hemo terapia da Universidade Federal de São Paulo

José O rla ndo Bordin - Pro feso r livre-do cente da disciplina de Hemato lo g ia e Hemo terapia da Universidade Federal de São Paulo

RESUMO

Contex to: Pacientes co m anemia falcifo rme recebem co m freqüência co ncentrado s de g ló bulo s vermelho s (CG V). Recentemente, nó s relatamo s que o risco de alo -imuniz ação eritro citária po r unidade transfundida em pacientes brasileiro s co m anemia falcifo rme é 1 ,1 5 %. Rela to de ca so: N ó s descrevemo s uma reação transfusio nal hemo lítica tardia (RTHT) apresentando -se co mo uma crise do lo ro sa em um paciente co m anemia falcifo rme.

Sao Paulo Med J/Rev Paul Med 1999; 117(1):38-9.

Sources of Funding: N o t declared

Conflict of interest: N o t declared

La st received: 2 8 September 1 9 9 7

Accepted: 1 1 September 1 9 9 8

Address for correspondence:

Jo sé O rlando Bo rdin

Disciplina de Hemato lo g ia e Hemo terapia Universidade Federal de São Paulo / EPM Rua Bo tucatu, 7 4 0