SAF y leucemia promielocítica aguda: un desafío terapéutico
cute promyelocitic leukemia (APL) is a subtype of leukemia which is associated with unique and distinctive coagulopathy. In the absence of treatment, it is rapidly fatal and even after initiation of therapy the major cause of early mortality is related to hemorrhagic complications. Antiphospholipid...
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Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2022
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| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/39037 |
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I10-R327-article-39037 |
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ojs |
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Universidad Nacional de Córdoba |
| institution_str |
I-10 |
| repository_str |
R-327 |
| container_title_str |
Revista de la Facultad de Ciencias Médicas de Córdoba |
| format |
Artículo revista |
| topic |
english spanish portugues |
| spellingShingle |
english spanish portugues Figueroa, RM Heller, V Alonso, C Savio, V Albiero, A Yorio, M Gobbi, C Alba, P SAF y leucemia promielocítica aguda: un desafío terapéutico |
| topic_facet |
english spanish portugues |
| author |
Figueroa, RM Heller, V Alonso, C Savio, V Albiero, A Yorio, M Gobbi, C Alba, P |
| author_facet |
Figueroa, RM Heller, V Alonso, C Savio, V Albiero, A Yorio, M Gobbi, C Alba, P |
| author_sort |
Figueroa, RM |
| title |
SAF y leucemia promielocítica aguda: un desafío terapéutico |
| title_short |
SAF y leucemia promielocítica aguda: un desafío terapéutico |
| title_full |
SAF y leucemia promielocítica aguda: un desafío terapéutico |
| title_fullStr |
SAF y leucemia promielocítica aguda: un desafío terapéutico |
| title_full_unstemmed |
SAF y leucemia promielocítica aguda: un desafío terapéutico |
| title_sort |
saf y leucemia promielocítica aguda: un desafío terapéutico |
| description |
cute promyelocitic leukemia (APL) is a subtype of leukemia which is associated with unique and distinctive coagulopathy. In the absence of treatment, it is rapidly fatal and even after initiation of therapy the major cause of early mortality is related to hemorrhagic complications. Antiphospholipid syndrome (APS) is a multisystem autoimmune disease associated with recurrent arterial and venous thrombosis and pregnancy loss. Recurrent thrombosis is the leading cause of mortality and long term anticoagulation therapy is required. Patients with antiphospholipid antibodies have an increased risk of developing hematological neoplasms and the risk of thrombosis. We report a case of APL who developed after years of diagnosis of thrombotic APS.
A 51 years old man was referred for hematology and rheumatology evaluation to our hospital. He had a medical history of primary APS diagnosed 20 years ago. He had 2 episodes of Myocardial infarction (when he was 30 and 40 years old respectively) with triple positivity of antiphospholipid antibodies (LA, aCL Ig G and B2GPI Ig G) and he has been treated with oral anticoagulation with warfarin plus aspirin with INR of 3, statins and hydroxycloroquine. He developed dyspnea and tiredness. Laboratory findings showed pancytopenia: Hot 32.1%, HB 8.4 grs%, WBC 1400, platelets 20.000. Antinuclear, ds-DNA, Sm, RNP, Ro and la antibodies were negative. Hepatitis B, C and syphilis serology was negative and complement levels were normal. CRP Sars-Cov2 was negative. Iron and folic acid levels were normal. BMP: 79% promyelocytes infiltration. Flow cytometry; 85% abnormal cells with immunophenotype of APL (CD34 -, HLA Dr -, CD117+, CD33 ++ (homogenous), CD13 ++ (heterogeneous), CD15 +/-, CD123 +, CD56 partial expression.). PML RAR ALFA (T 15,17): presence of PML RAR ALFA band. He started treatment with ATRA and he completed induction and seven cycles of consolidation therapy. He required platelets transfusions and Low weight molecular heparin bridge therapy for short periods. He had no hemorrhage neither thrombotic complications. 50 days after BM examination showed remission.
The association of APL and APS is extremely rare and a diagnostic and therapeutic challenge. The onset of cytopenia in APS require a clinical and laboratory workup and bone marrow (BM) examination to determine the cause and for appropriate patient management. Common causes should be exclude including development of SLE, infection, iron and folic deficiency or effect of medications. |
| publisher |
Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
| publishDate |
2022 |
| url |
https://revistas.unc.edu.ar/index.php/med/article/view/39037 |
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2024-09-03T21:04:01Z |
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I10-R327-article-390372024-04-15T16:14:45Z SAF y leucemia promielocítica aguda: un desafío terapéutico Figueroa, RM Heller, V Alonso, C Savio, V Albiero, A Yorio, M Gobbi, C Alba, P english spanish portugues cute promyelocitic leukemia (APL) is a subtype of leukemia which is associated with unique and distinctive coagulopathy. In the absence of treatment, it is rapidly fatal and even after initiation of therapy the major cause of early mortality is related to hemorrhagic complications. Antiphospholipid syndrome (APS) is a multisystem autoimmune disease associated with recurrent arterial and venous thrombosis and pregnancy loss. Recurrent thrombosis is the leading cause of mortality and long term anticoagulation therapy is required. Patients with antiphospholipid antibodies have an increased risk of developing hematological neoplasms and the risk of thrombosis. We report a case of APL who developed after years of diagnosis of thrombotic APS. A 51 years old man was referred for hematology and rheumatology evaluation to our hospital. He had a medical history of primary APS diagnosed 20 years ago. He had 2 episodes of Myocardial infarction (when he was 30 and 40 years old respectively) with triple positivity of antiphospholipid antibodies (LA, aCL Ig G and B2GPI Ig G) and he has been treated with oral anticoagulation with warfarin plus aspirin with INR of 3, statins and hydroxycloroquine. He developed dyspnea and tiredness. Laboratory findings showed pancytopenia: Hot 32.1%, HB 8.4 grs%, WBC 1400, platelets 20.000. Antinuclear, ds-DNA, Sm, RNP, Ro and la antibodies were negative. Hepatitis B, C and syphilis serology was negative and complement levels were normal. CRP Sars-Cov2 was negative. Iron and folic acid levels were normal. BMP: 79% promyelocytes infiltration. Flow cytometry; 85% abnormal cells with immunophenotype of APL (CD34 -, HLA Dr -, CD117+, CD33 ++ (homogenous), CD13 ++ (heterogeneous), CD15 +/-, CD123 +, CD56 partial expression.). PML RAR ALFA (T 15,17): presence of PML RAR ALFA band. He started treatment with ATRA and he completed induction and seven cycles of consolidation therapy. He required platelets transfusions and Low weight molecular heparin bridge therapy for short periods. He had no hemorrhage neither thrombotic complications. 50 days after BM examination showed remission. The association of APL and APS is extremely rare and a diagnostic and therapeutic challenge. The onset of cytopenia in APS require a clinical and laboratory workup and bone marrow (BM) examination to determine the cause and for appropriate patient management. Common causes should be exclude including development of SLE, infection, iron and folic deficiency or effect of medications. La leucemia promielocítica aguda (LPA) es un subtipo de leucemia que se asocia con una coagulopatía única y distintiva. En ausencia de tratamiento es rápidamente fatal e incluso después del inicio de la terapia. La principal causa de mortalidad temprana está relacionada con las complicaciones hemorrágicas. El síndrome antifosfolípido (SAF) es una enfermedad autoinmune multisistémica asociado a trombosis arterial y venosa recurrente y morbilidad obstétrica. La trombosis recurrente es la principal causa de mortalidad y la anticoagulación a largo plazo es la terapia requerida. Los pacientes con anticuerpos antifosfolípidos tienen un mayor riesgo de desarrollar neoplasias hematológicas y complicaciones trombóticas. Presentamos un caso de LPA, desarrollada luego de años de diagnóstico de SAF trombótico. Paciente varón de 51 años derivado a nuestro hospital para evaluación por hematología y reumatología. APP: SAF primario diagnosticado hace 20 años (2 episodios de IAM a los 30 y 40 años; triple positividad de anticuerpos antifosfolípidos: LA, aCL Ig G y B2GPI Ig G), que se encontraba anticoagulado con warfarina (INR de 3) además de aspirina, estatinas e hidroxicloroquina. Consulta en ese momento por disnea y fatiga. Laboratorio: Hb 8.4 grs% GB 1400/mm3 Plaq 20000/mm3. ANA, anti-DNA, Sm, Ro, La, RNP negativos. C3 y C4 normales. Ferremia y ácido fólico normal. Hisopado para Sars-Cov2 negativo. PMO: 79% infiltración de promielocitos. Citometría de flujo; 85% células anormales con inmunofenotipo de LPA (CD34-, HLA Dr-, CD117+, CD33++ (homogéneo), CD13++ (heterogéneo), CD15+/-, CD123+, expresión parcial de CD56).PML RAR ALFA (T 15,17): presencia de banda PML RAR ALFA. Inició tratamiento con ATRA, completó inducción y siete ciclos de terapia de consolidación. Requirió transfusiones de plaquetas. No presentó hemorragias, ni complicaciones trombóticas. La asociación de LPA y SAF es muy rara y representa un desafío diagnóstico y terapéutico. La aparición de citopenia en SAF requiere un estudio clínico, laboratorio y examen de MO para determinar la causa y definir el manejo terapéutico. Las causas comunes deben excluirse, tales como LES, infección, deficiencia de hierro y ácido fólico o toxicidad a medicamentos. Universidad Nacional Córdoba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2022-10-26 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion texto texto texto https://revistas.unc.edu.ar/index.php/med/article/view/39037 Revista de la Facultad de Ciencias Médicas de Córdoba.; Vol. 79 No. Suplemento JIC XXIII (2022): Suplemento JIC XXIII Revista de la Facultad de Ciencias Médicas de Córdoba; Vol. 79 Núm. Suplemento JIC XXIII (2022): Suplemento JIC XXIII Revista da Faculdade de Ciências Médicas de Córdoba; v. 79 n. Suplemento JIC XXIII (2022): Suplemento JIC XXIII 1853-0605 0014-6722 http://creativecommons.org/licenses/by-nc/4.0 |