ABSTRACT
The relationship between bullous pemphigoid (BP) and neoplasms has been debated in the medical literature. Although numerous case reports have described the coexistence of BP with various neoplasms, case-control studies have yielded conflicting results. We present the case of a male patient who developed BP shortly after being diagnosed with mycosis fungoides (MF). He was a 77-year-old man with a history of type 2 diabetes mellitus and hypertension who was diagnosed with MF. Subsequently, he developed blisters after sun exposure, and was diagnosed with BP through histopathology and direct immunofluorescence. The patient was treated with prednisone and methotrexate, with favorable evolution without recurrence of MF or appearance of new blisters. The association between cutaneous T-cell lymphoma and autoimmune blistering disease is rare, although similar cases have been reported, some associated with phototherapy. In this case, the onset of BP after sun exposure suggests a potential connection. The coexistence of BP and MF remains controversial, and this case highlights the importance of considering autoimmune blistering diseases in patients with oncohematological neoplasms.
La relación entre el penfigoide ampollar (PA) y las neoplasias ha sido objeto de debate en la literatura médica. Aunque numerosos informes de casos han descrito la coexistencia del PA con diversas neoplasias, estudios de casos y controles han arrojado resultados contradictorios. Presentamos el caso de un paciente masculino que desarrolló un PA poco después de ser diagnosticado con una micosis fungoide (MF). Se trata de un hombre de 77 años con antecedentes de diabetes mellitus tipo 2 e hipertensión arterial que fue diagnosticado con MF. Posteriormente, desarrolló ampollas después de una exposición solar, siendo diagnosticado con PA mediante histopatología e inmunofluorescencia directa. El paciente fue tratado con meprednisona y metotrexato, evolucionando favorablemente sin recurrencia de MF ni aparición de nuevas ampollas. La asociación entre un linfoma cutáneo de células T y una enfermedad ampollar autoinmune es rara, aunque han sido reportados casos similares, algunos asociados con fototerapia. En este caso la aparición del PA después de la exposición solar sugiere una conexión potencial. La coexistencia entre PA y MF sigue siendo controvertida, y este caso destaca la importancia de considerar enfermedades ampollares autoinmunes en pacientes con neoplasias oncohematológicas.
Subject(s)
Mycosis Fungoides , Pemphigoid, Bullous , Skin Neoplasms , Humans , Pemphigoid, Bullous/complications , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/etiology , Mycosis Fungoides/complications , Mycosis Fungoides/pathology , Mycosis Fungoides/diagnosis , Male , Aged , Skin Neoplasms/complications , Skin Neoplasms/pathology , Prednisone/therapeutic use , Methotrexate/therapeutic useABSTRACT
Resumen La leucemia/linfoma a células T del adulto (ATLL) es una enfermedad hematológica causada por el virus linfotrópico T humano tipo 1 (HTLV-1) que se desarrolla luego de 20 años de incubación, preferencialmente cuando la infección se adquiere por transmisión vertical. Este tiempo se reduce de 3 meses a 3 años cuando la transmisión del virus es por transfusión o trasplante de órganos. La ATLL aguda es de difícil diagnóstico por ser inusual y tener una rápida progresión a la muerte. En el Noroeste argentino, donde el virus es endémico, la ATLL es más frecuente, sin embargo, también se la detecta continuamente en el resto del país. El tratamiento de elección, en primera instancia, es el uso combinado de antivirales. Presentamos un caso de ATLL aguda desarrollada en un hombre de 59 años de Santiago del Estero a partir del cual se identificó transmisión intrafamiliar de la infección por HTLV-1.
Abstract Adult T-cell leukemia/lymphoma (ATLL) is an hematological disease caused by human T-cell lymphotropic virus type 1 (HTLV-1) that develops after 20 years of incubation preferentially when the infection is acquired by vertical transmission. In cases of transmission by transfusion or organ transplant, this time is reduced from 3 months to 3 years. Acute ATLL is difficult to diagnose because it is unusual and has a rapid progression to death. In the Argentine Northwest, where the virus is endemic, ATLL is more frequent, however it is also detected continuously in the rest of the country. The treatment of choice, in the first instance, is the combined use of antivirals. We present a case of acute ATLL developed in a 59-year-old man from Santiago del Estero from which intrafamilial transmission of HTLV-1 infection was identified.
Subject(s)
Humans , Male , Adult , Middle Aged , Human T-lymphotropic virus 1/genetics , HTLV-I Infections/diagnosis , Leukemia-Lymphoma, Adult T-Cell/diagnosis , T-LymphocytesABSTRACT
Adult T-cell leukemia/lymphoma (ATLL) is an hematological disease caused by human T-cell lymphotropic virus type 1 (HTLV-1) that develops after 20 years of incubation preferentially when the infection is acquired by vertical transmission. In cases of transmission by transfusion or organ transplant, this time is reduced from 3 months to 3 years. Acute ATLL is difficult to diagnose because it is unusual and has a rapid progression to death. In the Argentine Northwest, where the virus is endemic, ATLL is more frequent, however it is also detected continuously in the rest of the country. The treatment of choice, in the first instance, is the combined use of antivirals. We present a case of acute ATLL developed in a 59-year-old man from Santiago del Estero from which intrafamilial transmission of HTLV-1 infection was identified.
La leucemia/linfoma a células T del adulto (ATLL) es una enfermedad hematológica causada por el virus linfotrópico T humano tipo 1 (HTLV-1) que se desarrolla luego de 20 años de incubación, preferencialmente cuando la infección se adquiere por transmisión vertical. Este tiempo se reduce de 3 meses a 3 años cuando la transmisión del virus es por transfusión o trasplante de órganos. La ATLL aguda es de difícil diagnóstico por ser inusual y tener una rápida progresión a la muerte. En el Noroeste argentino, donde el virus es endémico, la ATLL es más frecuente, sin embargo, también se la detecta continuamente en el resto del país. El tratamiento de elección, en primera instancia, es el uso combinado de antivirales. Presentamos un caso de ATLL aguda desarrollada en un hombre de 59 años de Santiago del Estero a partir del cual se identificó transmisión intrafamiliar de la infección por HTLV-1.
Subject(s)
HTLV-I Infections , Human T-lymphotropic virus 1 , Leukemia-Lymphoma, Adult T-Cell , Adult , HTLV-I Infections/diagnosis , Human T-lymphotropic virus 1/genetics , Humans , Leukemia-Lymphoma, Adult T-Cell/diagnosis , Male , Middle Aged , T-LymphocytesABSTRACT
Abstract We retrospectively analyzed 570 adult patients who received allogeneic stem cell transplantation for malignant diseases. The outcomes were compared according to donor type. Most of the patients (60%) were transplanted for acute leukemia. Median follow-up was 1.6 years. Haploidentical allogeneic stem cell transplantation was more frequently performed for acute myeloid leukemia and in late stages than any other donor type. Non-relapse mortality at 100 days and one year for unrelated and haploidentical donors were similar, 19%-29% vs. 17%-28%, respectively. A significant better non-relapse mortality was observed for matched sibling donors (7%-15%; p < 0.001). Relapse rate was higher in haploidentical donors compared to matched sibling and unrelated donors (three year relapse rate 46%, 39%, 28%; respectively p < 0.001). Haploidentical donors resulted in lower three year progression-free survival and worse 3 year overall survival (32%; p < 0.001 and 42%; p < 0.001) compared with other donors (44% and 55% MSD, 40% and 42% UD, respectively). The incidence of grade II-IV acute graft-versus-host disease was higher in unrelated donors (51% unrelated, 35% haploidentical, 36% matched sibling; respectively; p = 0.001), with no difference in grades III-IV (p = 0.73) or in chronic graft-versus-host disease (p = 0.2) between groups. After multivariate analysis, haploidentical and unrelated donors remained negatively associated with non-relapse mortality (HR 1.95; 95% CI 1.10-3.20 and HR 2.70; 95% CI 1.63-4.46, respectively). Haploidentical donors were associated with a higher risk of relapse and worse overall survival. This analysis shows that haploidentical donors were associated with similar non-relpase mortality and higher relapse rates than unrelated donors. Better results in non-relapse mortality were observed for matched sibling donors.
Resumen Se efectuó un análisis retrospectivo de 570 pacientes adultos que recibieron un trasplante alogénico de precursores hematopoyéticos, comparando los resultados según el tipo de donante. La mediana de seguimiento fue de 1.6 años. El 60% de la población se trasplantó por leucemias agudas. Los trasplantes haploidénticos se hicieron en su mayoría en leucemia mieloide aguda y en estadios tardíos en comparación a otros donantes. La mortalidad libre de enfermedad al día +100 y a 1 año fue similar para los donantes no emparentados y haploidénticos (19% y 29% vs. 17% y 28%, respectivamente). Se obtuvieron mejores resultados con donantes relacionados idénticos (7% y 15%; p < 0.001). La recaída fue mayor en los donantes haploidénticos (tres años 46% haploidénticos, 39% relacionados idénticos, 28% no emparentados; p < 0.003). El trasplante con donante haploidéntico presentó una menor supervivencia libre de progresión y menor supervivencia global a tres años (32%; p < 0.001 y 42%; p < 0.001). La incidencia de enfermedad injerto contra huésped aguda fue mayor en no emparentados (51%, 35% haploidénticos, 36% relacionados idénticos; p = 0.001), sin diferencias en grados III-IV (p = 0.73) o en EICH crónica (p = 0.2). Los trasplantes con donante haploidéntico y no emparentado mantuvieron su asociación negativa con mortalidad libre de enfermedad (HR 1.95; 95%IC 1.10-3.20 y HR 2.70; 95%IC 1.63-4.46), en análisis multivariado. El trasplante haploidéntico se asoció a mayor recaída y a menor supervivencia global. Esta experiencia mostró similar mortalidad libre de enfermedad entre trasplantes con donantes haploidénticos y no emparentados. Los trasplantes relacionados idénticos mostraron menores tasas de mortalidad libre de enfermedad.
Subject(s)
Humans , Adult , Hematopoietic Stem Cell Transplantation , Graft vs Host Disease , Retrospective Studies , Bone Marrow Transplantation , Disease-Free Survival , SiblingsABSTRACT
We retrospectively analyzed 570 adult patients who received allogeneic stem cell transplantation for malignant diseases. The outcomes were compared according to donor type. Most of the patients (60%) were transplanted for acute leukemia. Median follow-up was 1.6 years. Haploidentical allogeneic stem cell transplantation was more frequently performed for acute myeloid leukemia and in late stages than any other donor type. Non-relapse mortality at 100 days and one year for unrelated and haploidentical donors were similar, 19%-29% vs. 17%-28%, respectively. A significant better non-relapse mortality was observed for matched sibling donors (7%-15%; p < 0.001). Relapse rate was higher in haploidentical donors compared to matched sibling and unrelated donors (three year relapse rate 46%, 39%, 28%; respectively p < 0.001). Haploidentical donors resulted in lower three year progression-free survival and worse 3 year overall survival (32%; p < 0.001 and 42%; p < 0.001) compared with other donors (44% and 55% MSD, 40% and 42% UD, respectively). The incidence of grade II-IV acute graft-versus-host disease was higher in unrelated donors (51% unrelated, 35% haploidentical, 36% matched sibling; respectively; p = 0.001), with no difference in grades III-IV (p = 0.73) or in chronic graft-versus-host disease (p = 0.2) between groups. After multivariate analysis, haploidentical and unrelated donors remained negatively associated with non-relapse mortality (HR 1.95; 95% CI 1.10-3.20 and HR 2.70; 95% CI 1.63-4.46, respectively). Haploidentical donors were associated with a higher risk of relapse and worse overall survival. This analysis shows that haploidentical donors were associated with similar non-relpase mortality and higher relapse rates than unrelated donors. Better results in non-relapse mortality were observed for matched sibling donors.
Se efectuó un análisis retrospectivo de 570 pacientes adultos que recibieron un trasplante alogénico de precursores hematopoyéticos, comparando los resultados según el tipo de donante. La mediana de seguimiento fue de 1.6 años. El 60% de la población se trasplantó por leucemias agudas. Los trasplantes haploidénticos se hicieron en su mayoría en leucemia mieloide aguda y en estadios tardíos en comparación a otros donantes. La mortalidad libre de enfermedad al día +100 y a 1 año fue similar para los donantes no emparentados y haploidénticos (19% y 29% vs. 17% y 28%, respectivamente). Se obtuvieron mejores resultados con donantes relacionados idénticos (7% y 15%; p < 0.001). La recaída fue mayor en los donantes haploidénticos (tres años 46% haploidénticos, 39% relacionados idénticos, 28% no emparentados; p < 0.003). El trasplante con donante haploidéntico presentó una menor supervivencia libre de progresión y menor supervivencia global a tres años (32%; p < 0.001 y 42%; p < 0.001). La incidencia de enfermedad injerto contra huésped aguda fue mayor en no emparentados (51%, 35% haploidénticos, 36% relacionados idénticos; p = 0.001), sin diferencias en grados III-IV (p = 0.73) o en EICH crónica (p = 0.2). Los trasplantes con donante haploidéntico y no emparentado mantuvieron su asociación negativa con mortalidad libre de enfermedad (HR 1.95; 95%IC 1.10-3.20 y HR 2.70; 95%IC 1.63-4.46), en análisis multivariado. El trasplante haploidéntico se asoció a mayor recaída y a menor supervivencia global. Esta experiencia mostró similar mortalidad libre de enfermedad entre trasplantes con donantes haploidénticos y no emparentados. Los trasplantes relacionados idénticos mostraron menores tasas de mortalidad libre de enfermedad.
Subject(s)
Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Adult , Bone Marrow Transplantation , Disease-Free Survival , Humans , Retrospective Studies , SiblingsABSTRACT
PURPOSE: To describe a bilateral macular detachment as the only sign of acute lymphoblastic leukemia relapse and prompt reversal with total body irradiation without ocular protection. OBSERVATIONS: We present the case of a 20-year-old patient, diagnosed with a high-risk phy-negative, pre-B acute lymphoblastic leukemia (ALL), with a positive MLL gene rearrangement. After a Berlin-Frankfurt-Munster-like regimen chemotherapy protocol and a first complete remission, ALL relapse was diagnosed, so he was commenced on a FlaG-Ida protocol (fludarabine, idarubicin, granulocyte-colony stimulating factor, and high-dose cytarabine). He achieved a second complete remission with positive minimal residual disease and was scheduled for urgent allogeneic bone marrow transplant.Five days before the conditioning regimen was initiated, the patient complained of visual loss in the left eye and then in the right eye. Ophthalmological evaluation showed a best corrected visual acuity of the right eye (OD) of 20/100 and of the left eye (OS) of 20/400. Optical coherence tomography (OCT) showed a bilateral serous sub-foveal detachment. The sub-foveal choroidal thickness was measured by enhanced depth imaging (EDI-OCT) and showed a significant increase (OD 836 µm and OS 1036 µm) compared with normal (average 310 µm). This choroidal thickness increase, associated with the serous macular detachment, was interpreted as a choroidal leukemic infiltration.A lumbar puncture with cytologic studies and flow cytometry was performed, showing no evidence of central nervous system (CNS) involvement of leukemia. CNS and orbital magnetic nuclear resonance imaging showed no pathology. No extramedullary involvement could be confirmed.Retinal fluorescein angiography showed multiple and diffuse leakage points (pinpoint pattern) within the macular area. This pattern reinforced our presumptive diagnosis, even though the lumbar puncture and flow cytometry were negative.The hematologist decided to proceed with the bone marrow transplant. A myeloablative conditioning regimen was delivered, based on total body irradiation (TBI) with a total dose of 12 Gy plus fludarabine 30 mg/m2 for five days. No ocular protection was used during TBI.Only 2 h after TBI commenced, the patient reported a significant improvement in his visual acuity. We confirmed 20/20 in both eyes. The OCT showed a dramatic decrease in the choroidal thickness measurement (OD 387 µm and OS 408 µm compared with 836 µm and 1036 µm measured before radiotherapy). CONCLUSIONS AND IMPORTANCE: Complete ophthalmological evaluation and EDI-OCT choroidal thickness measurement could be fundamental tools necessary to determine CNS involvement of ALL, even in cases with negative cerebrospinal fluid and brain imaging.
ABSTRACT
Introducción La disfunción ventricular izquierda es una complicación grave del tratamiento antineoplásico, con impacto desfavorable en la evolución clínica futura. El diagnóstico precoz de cardiotoxici-dad en pacientes que reciben quimioterapia podría ser de utilidad para definir una estrategia de prevención del deterioro de la función ventricular. Objetivo Analizar la utilidad de marcadores humorales [troponina T (TnT), BNP y NT-proBNP] y del strain bidimensional sistólico longitudinal (SBL) y radial (SBR) para la detección de disfunción ventricular sistólica en pacientes tratados con quimioterapia cardiotóxica. Material y métodos Se incluyeron forma prospectiva 36 pacientes, edad promedio (± DE) de 47 ± 16 años (42% hombres), con enfermedad neoplásica con masa miocárdica normal y fracción de eyección = 55% tratados con agentes antineoplásicos. Se efectuaron dosajes de marcadores humorales y ecocardiograma basales y al 2°, 3°, 4° y 6° mes posterior al inicio del tratamiento oncológico. Se consideró punto final (PF) a los 6 meses a la caída de la fracción de eyección según consenso internacional. Resultados Alcanzaron el PF 7 pacientes (19,4%). Se observaron los siguientes predictores relacionados con el PF: NT-proBNP 4° mes [PF positivo (G1) 152 ± 42 pg/ml vs. PF negativo (G2) 61 ± 38 pg/ml; p < 0,001], BNP 4° mes (G1 41 ± 12 pg/ml vs. G2 26 ± 11 pg/ml; p < 0,01), SBL 3er mes (G1 -16,3 ± 2,4% vs. G2 -19,6 ± 2,02%; p < 0,01) y 4° mes (G1 -15,9 ± 1,77% vs. G2 -19,9 ± 2,2%; p < 0,001) y SBR 4° mes (G1 46,4 ± 2,4% vs. G2 52 ± 3,4%; p < 0,001). Conclusiones El dosaje de péptidos natriuréticos y la medición del strain bidimensional sistólico longitudinal y radial fueron de utilidad para predecir disfunción sistólica ventricular de grado leve en pacientes tratados con quimioterapia.(AU)
Background Left ventricular dysfunction is a serious complication of antineoplastic treatment with unfavorable impact in future clinical outcome. Early diagnosis of cardiotoxicity in patients receiving chemotherapy might be useful to define a strategy for the prevention of ventricular function impairment. Objective The aim of this study was to analyze the usefulness of serum markers [troponin T (TnT), BNP and NT-proBNP] and two-dimensional longitudinal (LS) and radial (RS) strain to detect ventricular systolic dysfunction in patients treated with cardiotoxic chemotherapy. Methods Thirty six patients [average age (±SD) 47±16 years, 42% men], with neoplastic disease with normal myocardial mass and left ventricular ejection fraction (LVEF) =55% receiving chemotherapy treatment, were prospectively included. Assessment of serum markers and echocardiography were performed before chemotherapy and at 2, 3, 4 and 6 months after onset of cancer treatment. The 6-month cardiotoxicity endpoint (EP) was defined as reduced LVEF according to international consensus. Results Seven patients reached the EP (19.4%). Endpoint predictors were: NT-proBNP at 4 months (positive EP (G1): 152 ±42 pg/ml vs. negative EP (G2) 61±38 pg/ml; p <0.001), BNP at 4 months (G1 41±12 pg/ml vs. G2 26±11 pg/ml; p <0.01), two-dimensional LS at 3 months (G1 -16.3±2.4% vs. G2 19.6±2.02%; p <0.01) and 4 months (G1 -15.9±1.77% vs. G2 19.9±2.2%; p <0.001), and two-dimensional RS at 4 months (G1 46.4±2.4% vs. G2 52±3.4%; p <0.001). Conclusions Natriuretic peptides and two-dimensional LS and RS were useful to predict mild ventricular systolic dysfunction in chemotherapy-treated patients.(AU)
ABSTRACT
Introducción La disfunción ventricular izquierda es una complicación grave del tratamiento antineoplásico, con impacto desfavorable en la evolución clínica futura. El diagnóstico precoz de cardiotoxici-dad en pacientes que reciben quimioterapia podría ser de utilidad para definir una estrategia de prevención del deterioro de la función ventricular. Objetivo Analizar la utilidad de marcadores humorales [troponina T (TnT), BNP y NT-proBNP] y del strain bidimensional sistólico longitudinal (SBL) y radial (SBR) para la detección de disfunción ventricular sistólica en pacientes tratados con quimioterapia cardiotóxica. Material y métodos Se incluyeron forma prospectiva 36 pacientes, edad promedio (± DE) de 47 ± 16 años (42% hombres), con enfermedad neoplásica con masa miocárdica normal y fracción de eyección = 55% tratados con agentes antineoplásicos. Se efectuaron dosajes de marcadores humorales y ecocardiograma basales y al 2°, 3°, 4° y 6° mes posterior al inicio del tratamiento oncológico. Se consideró punto final (PF) a los 6 meses a la caída de la fracción de eyección según consenso internacional. Resultados Alcanzaron el PF 7 pacientes (19,4%). Se observaron los siguientes predictores relacionados con el PF: NT-proBNP 4° mes [PF positivo (G1) 152 ± 42 pg/ml vs. PF negativo (G2) 61 ± 38 pg/ml; p < 0,001], BNP 4° mes (G1 41 ± 12 pg/ml vs. G2 26 ± 11 pg/ml; p < 0,01), SBL 3er mes (G1 -16,3 ± 2,4% vs. G2 -19,6 ± 2,02%; p < 0,01) y 4° mes (G1 -15,9 ± 1,77% vs. G2 -19,9 ± 2,2%; p < 0,001) y SBR 4° mes (G1 46,4 ± 2,4% vs. G2 52 ± 3,4%; p < 0,001). Conclusiones El dosaje de péptidos natriuréticos y la medición del strain bidimensional sistólico longitudinal y radial fueron de utilidad para predecir disfunción sistólica ventricular de grado leve en pacientes tratados con quimioterapia.
Background Left ventricular dysfunction is a serious complication of antineoplastic treatment with unfavorable impact in future clinical outcome. Early diagnosis of cardiotoxicity in patients receiving chemotherapy might be useful to define a strategy for the prevention of ventricular function impairment. Objective The aim of this study was to analyze the usefulness of serum markers [troponin T (TnT), BNP and NT-proBNP] and two-dimensional longitudinal (LS) and radial (RS) strain to detect ventricular systolic dysfunction in patients treated with cardiotoxic chemotherapy. Methods Thirty six patients [average age (±SD) 47±16 years, 42% men], with neoplastic disease with normal myocardial mass and left ventricular ejection fraction (LVEF) =55% receiving chemotherapy treatment, were prospectively included. Assessment of serum markers and echocardiography were performed before chemotherapy and at 2, 3, 4 and 6 months after onset of cancer treatment. The 6-month cardiotoxicity endpoint (EP) was defined as reduced LVEF according to international consensus. Results Seven patients reached the EP (19.4%). Endpoint predictors were: NT-proBNP at 4 months (positive EP (G1): 152 ±42 pg/ml vs. negative EP (G2) 61±38 pg/ml; p <0.001), BNP at 4 months (G1 41±12 pg/ml vs. G2 26±11 pg/ml; p <0.01), two-dimensional LS at 3 months (G1 -16.3±2.4% vs. G2 19.6±2.02%; p <0.01) and 4 months (G1 -15.9±1.77% vs. G2 19.9±2.2%; p <0.001), and two-dimensional RS at 4 months (G1 46.4±2.4% vs. G2 52±3.4%; p <0.001). Conclusions Natriuretic peptides and two-dimensional LS and RS were useful to predict mild ventricular systolic dysfunction in chemotherapy-treated patients.