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Transfusion of patients with autoimmune When the patient has a broadly reactive The management of such patients is one of
hemolytic anemia (AIHA) presents a unique autoantibody, as is generally true, the the most critical responsibilities of the
set of potential problems. transfusion service is likely to find that all transfusion medicine service in conjunction
units of red blood cells (RBCs) are with clinicians who have primary care
incompatible. responsibility.
After reading this article, the reader should understand the role of the Blood banking exam 60501 questions and corresponding answer form
clinician and the laboratory in dealing with emergency transfusions for are located after the CE update exam on p. 49.
autoimmune hemolytic anemia.
It is quite unusual for a patient to have AIHA of life-threat- benefit.1,9,10 Therefore, the indications for transfusion in pa-
ening severity when first seen. However, uncertainty as to appro- tients with AIHA are not significantly different than for simi-
priate management, both in the laboratory and on the clinical larly anemic patients without AIHA, as long as appropriate
services, too often leads to delays which allow the development compatibility procedures are performed to detect and identify
of severe and even life-threatening anemia, which then becomes RBC alloantibodies.1,3-5,9,11
a medical emergency.
Prompt evaluation and management will generally avoid
the need of transfusing the patient on an emergency basis. Trans- Communication Between the Clinician and
fusion of patients with autoimmune hemolytic anemia (AIHA) the Transfusion Service
presents a unique set of potential problems.1-5 Perhaps one of the Responsibilities of the Clinician. A discussion between
most common mistakes in management is the reluctance to the attending physician and the transfusion service should
transfuse patients with AIHA because of uncertainty regarding take place as soon as it is evident that a patient with AIHA is
the safety and effectiveness of RBC units that are “incompatible” being considered for transfusion.1,2,12,13 The clinician should
because of the presence of a RBC autoantibody. Examples of indicate the urgency of the transfusion and discuss with the
patients being denied transfusion in spite of clear indications transfusion service personnel the time required for the more
have been reported by Conley and colleagues.6,7 These authors7 detailed than usual serologic studies that will be necessary.
described 5 patients with AIHA and reticulocytopenia who de- The clinician should also discuss the compatibility tests to be
veloped life-threatening anemia but who were not transfused undertaken by the laboratory using the outline of compatibil-
because their physicians were concerned that compatible blood ity test procedures provided below as a guide to adequate pre-
could not be obtained. This was true even though the patients’ transfusion testing, and seek assurance that appropriate testing
hematocrits were at a level of 8% to 10%. After transfer to a is to be performed.
tertiary care medical center, the patients were promptly trans- Responsibilities of the Transfusion Service. In some in-
fused, a measure that the authors felt was unquestionably lifesav- stances, it will be the responsibility of the transfusion service to
ing. initiate the communication since the diagnosis of AIHA may
first be made during compatibility testing for a requested trans-
fusion. In any case, the transfusion service should feel obligated
Indications for Transfusion in AIHA to supply the clinician with information about the compatibility
Hemolytic transfusion reactions are expected to occur test procedures performed. After appropriate testing, the clini-
when incompatibility is due to clinically important alloanti- cian should be assured that transfused RBCs are unlikely to
bodies.8 However, experience indicates that when incompati- cause an acute hemolytic transfusion reaction even though the
bility is due only to the presence of a RBC autoantibody, acute RBCs cannot be expected to survive normally because of the
reactions are unlikely, survival of transfused RBCs is generally patient’s autoantibody. The attending physician can then pro-
about as good as survival of the patient’s own RBCs, and ceed to make a decision regarding transfusion on the basis of the
transfusion can be expected to cause significant temporary clinical need.
For example, performing an adsorption using a Jk(a-) cell, “Least Incompatible” Units
of a serum containing a warm autoantibody and an anti-Jka al- The term “least incompatible” unit seems to be used very
loantibody will remove the autoantibody but not the anti-Jka. By frequently in transfusion services (at least in the United States),
selecting 2 or 3 samples of RBCs of various phenotypes for the although it is not defined in the medical literature and is used
alloadsorption procedure, alloantibodies that are responsible for differently by different transfusion medicine professionals.1,2,12
almost all clinically important hemolytic transfusion reactions Generally, the term is applied to the procedure by which a trans-
can be detected.1,15,16 fusion service selects a unit of RBCs for transfusion to a patient
with AIHA by merely testing the reactivity of the patient’s au-
toantibody against a number of ABO compatible units, and se-
Transfusion of Phenotypically Matched RBC lecting the unit that reacts least strongly.22
When extended phenotyping of the patient’s RBCs is per- Selecting “least incompatible” units must not be considered
formed, it is possible to determine which alloantibodies a patient an acceptable alternative to the techniques described above for
could develop as a result of previous transfusions or pregnancies. selecting donor units for transfusion of patients with AIHA.
For example, if a patient is Jk(a+), it is impossible to develop an This process as the sole means of selecting RBCs for transfusion
anti-Jka alloantibody. Transfusion of RBCs that are selected on of such patients will not reliably detect alloantibodies and is un-
the basis of the patient’s extended phenotype can provide a signif- acceptable in modern day transfusion medicine. This is a dan-
icant measure of safety,18 but some caveats and precautions must gerous practice and should be abandoned, except in extremely
be stressed.3 urgent settings in which there is not time to perform adequate
To provide adequate safety, typing must be performed for serologic tests.
numerous RBC antigens (eg, D, C, E, c, e, K, Jka, Jkb, Fya, Fyb, It is true that there is no evident disadvantage to selecting
S, and s). However, determining the extended phenotype is the unit that reacts least strongly from among those selected for
technically difficult when the patient has a positive direct transfusion on the basis of adsorption tests or extended RBC
antiglobulin (Coombs’) test and may be impossible in a signifi- phenotyping. However, after selecting a number of units of
cant percentage of patients with warm antibody AIHA even RBCs for transfusion on the basis of these procedures, it is not
when attempted by the most skilled technologists.18 Whether likely that significant added benefit will be gained for testing
implementation of this approach is cost-effective and feasible at these units for the “least incompatible.” Further, the use of the
many hospitals and blood centers has not been determined. If term in discussion with clinicians can lead only to confusion and
the intention is to emphasize providing phenotype-matched a lack of confidence in the safety of units selected by the transfu-
units, it must be determined that the blood supplier could read- sion service for transfusion to a patient with AIHA. This, in
ily provide such units, and it must be recognized that adsorp- turn, may lead to avoiding transfusion in a situation where
tion studies will be required in cases were the patient’s RBCs transfusion is needed. The use of the term “least incompatible”
cannot be phenotyped. unit should be discarded.1,2,12
only 1 to 3 adsorptions of the patient’s serum with the 9. Garratty G, Petz LD. Transfusing patients with autoimmune haemolytic
patient’s (ZZAP-treated) RBCs. anaemia. Lancet. 1993;341:1220.
The most time-consuming procedure, which may take 10. Salama A, Berghofer H, Mueller-Eckhardt C. Red blood cell transfusion in
warm-type autoimmune haemolytic anaemia. Lancet. 1992; 340:1515-1517.
about 4 hours, is allogeneic adsorption, which is indicated if the
11. Petz LD. Blood transfusion in hemolytic anemias. Immunohem. 1999;15:15-
patient has been transfused recently or if the patient’s RBCs are 23.
not available for autoadsorption. In order to expedite such test- 12. Petz LD. "Least incompatible" units for transfusion in autoimmune hemolytic
ing, transfusion services should plan ahead and have available anemia: should we eliminate this meaningless term? A commentary for
several mL of packed RBCs of each type that is needed. For ex- clinicians and transfusion medicine professionals. Transf. 2003;43:1503-1507.
ample, one may use 3 samples of allogeneic RBCs, 1 rr, 1 R1R1 13. Jefferies LC. Transfusion therapy in autoimmune hemolytic anemia.
and 1 R2R2; 1 sample should be Jk(a-) and 1 should be Jk(b-). Hematology/Oncology Clinics of N Am. 1994;8:1087-1104.
The RBCs can be stored in the frozen state or, if their need is 14. Petz LD. Blood transfusion in acquired hemolytic anemias. In: Petz LD,
Swisher SN, Kleinman S, et al, editors. Clinical Practice of Transfusion
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up to 6 months.1 LM 15. Petz LD, Branch DR. Serological tests for the diagnosis of immune hemolytic
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