A CASE OF AUTOANTI-CE (RH7) MASKED IN A PANAGGLUTINATION DURING A HEMOLYTIC CRISIS OF A PATIENT WITH CHRONIC LYMPHOCITIC LEUKEMIA
- 1Hospital Central de las Fuerzas Armadas, Uruguay
Abstract
Introduction: Chronic lymphocitic leukemias (CLL) are B cell malignancies that frequently display the production of autoantibodies that crossreact with the patient's own red cells, which in turn can cause an hemolytic crisis. The Rh blood group has been observed to be a predilect target for these autoantibodies. Panagglutinating autoantibodies or directed against the main Rh antigens (C, c, E and e) are fairly common. Antibodies against compound Rh antigens (Ce, cE, CE and ce) are rarely detected, since they are difficult to diagnose due the need of special cells for confirmation. We present the case of an 80 years-old male patient who developed an autoanti-Ce (RH7) masked by a panagglutination, detected first during the course of an hemolytic crisis. Objective: To describe the case of an autoanti-Ce (RH7) and the laboratory workup needed to its diagnosis. Case description: In 2025, a 79 years-old male patient diagnosed with CLL and under venetoclax treatment presented to our hospital with a one-week history of fatigue, chest pain and palpitations. On admission, he had jaundice, dark urine, hemoglobin of 5,8 g/dL and falling, bilirrubin 8,92 mg/dL and LDH of 2144 U/L, with no prior history of transfusions. The patient was group A RhD+, C+ c- E- e+ K- with a positive DAT (3+), a panagglutinating warm autoantibody (2+) diagnosed as an autoanti-e-like and a distinct autoanti-C (3+). A venetoclax-related autoimmune hemolytic anemia was suspected, and the patient started methilprednisolone, and was later switched to prednisone. He received 7 total units of packed red cells matched for Rh and K, well tolerated. In 2026 the patient returned and had developed immune thrombocytopenia with no response to corticoids or intravenous immunoglobulin. He was under treatment with zanubrutinib, had an hemoglobin of 10,5 g/dL, a platelet count of 6000/uL and no clinical or paraclinical elements of hemolysis. A second sample was received for an immunohematology update. He continued to present a positive DAT (3+) and the warm autoantibody that caused a panagglutination (2+) but this time the autoanti-C could not be detected. A triple differential alloadsortion was performed with our institution's red cell panel, using R1R1, R2R2 and rr cells with complementary antigens between them, to rule out the presence of alloantibodies. The adsorbed aliquots were tested with our 6-cell in-house panel, and an anti-C was found that failed to react with the RzR2 cell (C+ e- RH7-), which made us suspect an anti-RH7. Since the patient is R1R1 (therefore RH7+), this would also be an autoantibody. To complete the diagnosis, two specific tests were added: we tested the adsorbed sample with a 3-cell RzR2 (RH7-) in-house panel, and we performed one round of adsortions with our RzR2 cell. The RzR2 panel came back negative, and our RzR2 (C+ e-) cell failed to remove the ''anti-C'', while exhausting the other autoantibody, confirming the anti-RH7 specificity. Discussion: In the world of autoantibodies, those against the main Rh antigens are common. Antibodies against compound Rh antigens are less understood and are hard to even suspect, and the immunohematologic profile of these compound antigens its still not completely understood. Hemolytic events due to these antibodies are scarcely reported. The use of cells from the in-house panel produced from our own institution proved to be the key to this study. To the best of our knowledge, this is the first known case in our country.
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BibTeX
@article{lopez2026case,
title = {{A CASE OF AUTOANTI-CE (RH7) MASKED IN A PANAGGLUTINATION DURING A HEMOLYTIC CRISIS OF A PATIENT WITH CHRONIC LYMPHOCITIC LEUKEMIA}},
author = {Yuro LóPEZ and Macarena CAVALLERI and Lorena GUIGOU and Johana GóNGORA and Fabián ESTAVILLO and Rosario GóMEZ REARDEN},
journal = {Hematology, Transfusion and Cell Therapy},
year = {2026},
month = oct,
doi = {10.1016/j.htct.2026.106960},
url = {https://doi.org/10.1016/j.htct.2026.106960},
note = {CC BY},
}
RIS
TY - JOUR
TI - A CASE OF AUTOANTI-CE (RH7) MASKED IN A PANAGGLUTINATION DURING A HEMOLYTIC CRISIS OF A PATIENT WITH CHRONIC LYMPHOCITIC LEUKEMIA
AU - Yuro LóPEZ
AU - Macarena CAVALLERI
AU - Lorena GUIGOU
AU - Johana GóNGORA
AU - Fabián ESTAVILLO
AU - Rosario GóMEZ REARDEN
PY - 2026
DA - 2026/10/23
JO - Hematology, Transfusion and Cell Therapy
DO - 10.1016/j.htct.2026.106960
UR - https://doi.org/10.1016/j.htct.2026.106960
AB - Introduction: Chronic lymphocitic leukemias (CLL) are B cell malignancies that frequently display the production of autoantibodies that crossreact with the patient's own red cells, which in turn can cause an hemolytic crisis. The Rh blood group has been observed to be a predilect target for these autoantibodies. Panagglutinating autoantibodies or directed against the main Rh antigens (C, c, E and e) are fairly common. Antibodies against compound Rh antigens (Ce, cE, CE and ce) are rarely detected, since they are difficult to diagnose due the need of special cells for confirmation. We present the case of an 80 years-old male patient who developed an autoanti-Ce (RH7) masked by a panagglutination, detected first during the course of an hemolytic crisis. Objective: To describe the case of an autoanti-Ce (RH7) and the laboratory workup needed to its diagnosis. Case description: In 2025, a 79 years-old male patient diagnosed with CLL and under venetoclax treatment presented to our hospital with a one-week history of fatigue, chest pain and palpitations. On admission, he had jaundice, dark urine, hemoglobin of 5,8 g/dL and falling, bilirrubin 8,92 mg/dL and LDH of 2144 U/L, with no prior history of transfusions. The patient was group A RhD+, C+ c- E- e+ K- with a positive DAT (3+), a panagglutinating warm autoantibody (2+) diagnosed as an autoanti-e-like and a distinct autoanti-C (3+). A venetoclax-related autoimmune hemolytic anemia was suspected, and the patient started methilprednisolone, and was later switched to prednisone. He received 7 total units of packed red cells matched for Rh and K, well tolerated. In 2026 the patient returned and had developed immune thrombocytopenia with no response to corticoids or intravenous immunoglobulin. He was under treatment with zanubrutinib, had an hemoglobin of 10,5 g/dL, a platelet count of 6000/uL and no clinical or paraclinical elements of hemolysis. A second sample was received for an immunohematology update. He continued to present a positive DAT (3+) and the warm autoantibody that caused a panagglutination (2+) but this time the autoanti-C could not be detected. A triple differential alloadsortion was performed with our institution's red cell panel, using R1R1, R2R2 and rr cells with complementary antigens between them, to rule out the presence of alloantibodies. The adsorbed aliquots were tested with our 6-cell in-house panel, and an anti-C was found that failed to react with the RzR2 cell (C+ e- RH7-), which made us suspect an anti-RH7. Since the patient is R1R1 (therefore RH7+), this would also be an autoantibody. To complete the diagnosis, two specific tests were added: we tested the adsorbed sample with a 3-cell RzR2 (RH7-) in-house panel, and we performed one round of adsortions with our RzR2 cell. The RzR2 panel came back negative, and our RzR2 (C+ e-) cell failed to remove the ''anti-C'', while exhausting the other autoantibody, confirming the anti-RH7 specificity. Discussion: In the world of autoantibodies, those against the main Rh antigens are common. Antibodies against compound Rh antigens are less understood and are hard to even suspect, and the immunohematologic profile of these compound antigens its still not completely understood. Hemolytic events due to these antibodies are scarcely reported. The use of cells from the in-house panel produced from our own institution proved to be the key to this study. To the best of our knowledge, this is the first known case in our country.
SN - 2531-1379
SN - 2531-1387
ER -