Efficacy and Safety of Allogeneic Hematopoietic Stem Cell Transplantation with Decitabine-containing Regimen in Myelodysplastic Syndromes and Myelodysplastic Syndromes Transformed Acute Myeloid Leukemia / 中国实验血液学杂志

OBJECTIVE@#To evaluate the efficacy and safety of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with decitabine (Dec)-conditioning regimen in the treatment of myelodysplastic syndrome (MDS) and MDS transformed acute myeloid leukemia (MDS-AML).@*METHODS@#The characteristics and efficacy data of 93 patients with MDS and MDS-AML who received allo-HSCT in our center from April 2013 to November 2021 were retrospectively analyzed. All patients were administered by myeloablative conditioning regimen containing Dec (25 mg/m2 /d×3 d).@*RESULTS@#Among the 93 patients, 63 males and 30 females, were diagnosed as MDS（n ＝77）, MDS-AML（n ＝16）. The incidence of I/II grade regimen-related toxicity (RRT) was 39.8%, and III grade RRT was only found in 1 patient (1%). Neutrophil engraftment was successful in 91 (97.8%) patients after a median neutrophil engraftment time of 14 (9-27) days; Successful platelet engraftment was achieved in 87 (93.5%) patients, with a median engraftment time of 18 (9-290) days. The incidence of acute graft versus host disease（aGVHD） and grade III-IV aGVHD was 44.2% and 16.2%, respectively. The incidence of chronic graft versus host disease（cGVHD） and moderate-to-severe cGVHD was 59.5% and 37.1%, respectively. Of the 93 patients, 54 (58%) developed posttransplant infections, among which lung infection (32.3%) and bloodstream infection (12.9%) were the most common. The median follow-up after transplantation was 45 (0.1-108) months. The 5-year overall survival (OS) rate, disease-free survival (DFS) rate, treatment-related mortality, and cumulative incidence of relapse were 72.7%, 68.4%, 25.1%, and 6.5%, respectively. And the 1-year graft-versus-host disease/relapse-free survival rate was 49.3%. The patients in different group of relative high-risk prognostic scoring or low-risk prognostic scoring, with or without poor-risk mutation(s), with mutations number ≥3 or <3 had similar 5-year OS rate (more than 70%). Multivariate analysis showed that the incidence of grade III-IV aGVHD was the independent risk factor affecting OS（P =0.008）and DFS (P =0.019).@*CONCLUSION@#Allo-HSCT with Dec-conditioning regimen is feasible and effective in the treatment of patients with MDS and MDS-AML, especially those in high prognostic risk and with poor-risk mutations.

Clinical Characteristics and Prognosis of Patients with Hematological Malignancies Superimposed with Solid Tumors / 中国实验血液学杂志

OBJECTIVE@#To investigate the clinical characteristics and prognosis of hematological malignancies superimposed patients with solid tumors.@*METHODS@#The clinical data of 30 patients with more than two kinds of malignancy (the second is hematological malignancy) from October 2011 to October 2020 in Department of Hematology, Jiangning Hospital Affiliated to Nanjing Medical University were collected and analyzed retrospectively. The overall survival time was used as the prognostic evaluation standard, and the survival of patients were analyzed by KaplanMeier method. Logrank test and Cox regression model were used to carry out univariate and multivariate retrospective analysis on clinical and laboratory parameters of 30 patients.@*RESULTS@#Among 30 cases, 20 were male, 10 were female, the median age of onset of the second tumor was 70 years old. The common types of the secondary hematological malignancies to solid tumors are myelodysplastic syndrome, acute myeloid leukemia, multiple myeloma. Univariate analysis showed that patients' gender, age, type of solid tumors, the onset of interval between two kinds of tumor, chromosome karyotype were not related to do with the patients' overall survival time. Type of hematologic disease, ECOG score were associated with patients' overall survival time, and the multivariate analysis showed that the type of hematologic disease and ECOG score were independent risk factors for patients with poor prognosis.@*CONCLUSION@#Patients superimposed with solid tumors complicated with myelodysplastic syndrome or acute leukemia and ECOG score ≥3 have poor prognosis and shorter overall survival time, which are independent risk factors influencing the prognosis. Bone marrow injury, immune dysfunction and genetic susceptibility after chemoradiotherapy may be the main causes of these diseases.

Síndromes mielodisplásicos y sistema inmunitario / Myelodysplastic Syndrome and Immune System

Introducción: Los síndromes mielodisplásicos constituyen un grupo heterogéneo de desórdenes hematológicos clonales adquiridos, que afectan la célula madre. Se caracterizan morfológicamente por: hematopoyesis ineficaz, citopenias periféricas progresivas, displasia en uno o más linajes celulares y tendencia evolutiva a leucemia aguda. Los avances recientes en la comprensión de los mecanismos genéticos y moleculares de los síndromes mielodisplásicos, han revelado la asociación entre alteraciones inmunológicas y las mutaciones recurrentes. Las células de la respuesta inmune innata y adaptativa, así como diversos mediadores solubles liberados por ellas, pueden establecer una respuesta antitumoral protectora o, por el contrario, inducir eventos de inflamación crónica que favorezcan la promoción y progresión de esta enfermedad. Objetivos: Resumir los conocimientos actuales de la relación sistema inmune-síndromes mielodisplásicos, enfatizando en las células inmunes del microambiente de la médula ósea y su importancia en la clínica de la enfermedad. Métodos: Se realizó investigación bibliográfica-documental acerca del tema. Se consultaron las bases de datos Scielo y Pubmed. Conclusiones: La comprensión de la función dual que ejerce el sistema inmune en los síndromes mielodisplásicos, constituye un desafío y son necesarios estudios clínicos rigurosos para poder establecer el valor de la manipulación del sistema inmune como una forma posible de tratamiento de esta enfermedad(AU)

Introduction: Myelodysplastic syndromes (MDS) constitute a heterogeneous group of acquired clonal hematological disorders that affect the stem cell. These are characterized morphologically and clinically by: ineffective hematopoiesis, progressive peripheral cytopenia, dysplasia in one or more cell lineages, in most of cases and evolutionary tendency to acute leukemia. Recent advances in understanding the genetic and molecular mechanisms of MDS have revealed the association between immunological alterations and recurrent mutations. Cells of the innate and adaptive immune response, as well as various soluble mediators released by them, can establish a protective antitumor response or, on the contrary, induce events of chronic inflammation that favor the promotion and progression of this disease. Objective: To summarize the current knowledge of the immune system-MDS relationship, emphasizing the immune cells of the bone marrow microenvironment and their importance in the clinic of the disease. Methods: A bibliographic-documentary research was carried out on the subject. The Scielo and Pubmed databases were consulted. Conclusions: Understanding the dual role of the immune system in MDS constitutes a challenge and rigorous clinical studies are necessary to establish the value of manipulating the immune system as a possible form of treatment of this disease(AU)

Perfil nutricional de pacientes onco-hematológico internados em um Hospital Especializado em câncer em São Luís - MA / Nutritional Profile of Onco-hematological Patients Hospitalized in a Cancer Specialized Hospital in São Luís - MA

Objetivo: Avaliar o perfil nutricional de pacientes onco-hematológicos internados em um hospital especializado em câncer em São Luís - MA. Metodologia: Estudo transversal, retrospectivo, analítico, com coleta de dados secundária, envolvendo pacientes com idade mínima de 18 anos, de ambos os gêneros e que tenham sido submetidos a pelo menos uma ASG-PPP (Avaliação Subjetiva Global Produzida Pelo Paciente). Os dados foram coletados em registros do Serviço de Nutrição e Dietética do hospital. Analisaram-se dados demográficos (gênero e idade), clínicos (diagnóstico) e nutricionais: Índice de massa corporal (IMC), circunferên-cia braquial (CB), prega cutânea tricipital (PCT), circunferência muscular do braço (CMB) e ASG-PPP. As análises foram realizadas no programa estatístico Stata® 13.0. O nível de significância utilizado para os testes foi de p<0,05. Resultados: Foram avaliados 330 pacientes, onde foi constatado que 67,58% eram de adultos e 32,42% de idosos, com maior frequência do sexo masculino, com 60,30%. Ocorreu maior incidência de leucemia (58,48%), seguidos de linfomas (24,85%), mieloma múltiplo (13,33%) e síndrome mielodisplásica (3,3%). Na avaliação do estado nutricional os resultados mostraram que a ASG-PPP detectou maior número de pacientes com algum grau de desnutrição do que outros indicadores (93,94%), seguido pela PCT (65,76%), CMB (53,64%), CB (45,45%) e IMC (14,87%). De acordo com o IMC, foi encontrado maior incidência de eutrofia, correspondendo a 57,27% da amostra. Conclu-são: Diante do que foi encontrado, destaca-se que a desnutrição é um aspecto de extrema importância a ser considerado no tratamento de pacientes onco-hematológicos, visto que pode interferir diretamente no prognóstico da doença. (AU)

Objective: To evaluate the nutritional profile of onco-hematological patients admitted to a specialized cancer hospital in São Luís - MA. Methodology: Cross-sectional, retrospective, analytical study, with secondary data collection, involving patients aged at least 18 years, of both genders and having undergone at least one ASG-PPP (Subjective Global Assessment Produced by the Patient). Data were collected from records of the Hospital's Nutrition and Dietetics Service. Demographic (gender and age), clinical (diagnostic) and nutritional: Body Mass Index (BMI), Brachial Circumference (CB), Tricipital Skinfold (PCT), Muscular Arm Circumference (CMB) and ASG-PPP data were analyzed. The analyses were performed using the Stata® 13.0 statistical program. The level of significance used for the tests was p<0.05. Results: 330 patients were evaluated, in which it was found that 67.58% are adults and 32.42% are elderly, with a higher frequency of males with 60.30%. There was a higher incidence of Leukemia (58.48%), followed by Lymphomas (24.85%), Multiple Myeloma (13.33%) and Myelodysplastic Syndrome (3.3%). In the assessment of nutritional status, the results showed that ASG-PPP detected a greater number of patients with some degree of malnutrition than other indicators (93.94%), followed by PCT (65.76%), CMB (53.64%), CB (45.45%) and BMI (14.87%). According to the BMI, a higher incidence of eutrophy was found, which corresponds to 57.27% of the sample. Conclusion: In view of what was found, it is highlighted that malnutrition is an extremely important aspect to be considered in the treatment of onco-hematological patients, since it can directly interfere in the prognosis of the disease. (AU)

Myelodysplasia-Related Features of Acute Myeloid Leukemia Evolving From Philadelphia-Negative Myeloproliferative Neoplasms

No abstract available.

Síndrome de Sweet asociado a síndrome mielodisplásico / Sweet syndrome associated with myelodysplastic syndrome

El síndrome de Sweet o dermatosis neutrofílica febril aguda es una enfermedad sistémica de etiología desconocida, caracterizada por aparición brusca de fiebre y lesiones cutáneas, asociadas con leucocitosis y neutrofilia. Puede ser idiopático o estar asociado a Enfermedades hematológicas, procesos inflamatorios, infecciones, fármacos o embarazo. Las mielodisplasias son trastornos hematológicos caracterizados por una o más citopenias secundarias a disfunción de la médula ósea. Presentamos el caso de un paciente de 81 años con síndrome de Sweet y posterior diagnóstico de síndrome mielodisplásico, con buena respuesta al tratamiento corticoideo y resolución de las lesiones cutáneas. (AU)

Sweet's syndrome or acute febrile neutrophilic dermatosis is a systemic disease of unknown etiology characterized by sudden onset of painful skin lesions; predominance of polymorphonuclear leukocytosis. It can be associated with hematologic malignancies, idiopathic, parainflammatory, secondary to drugs and pregnancy. Myelodysplasias are haematological disorders characterized by one or more cytopenias secondary to bone marrow dysfunction. We report the case of a patient of 81 years with Sweet syndrome with subsequent diagnosis of myelodysplastic syndrome with good response to corticosteroid treatment and resolution of the skin lesions. (AU)

Endoscopic Submucosal Dissection for Early Gastric Cancer in a Patient with Myelodysplastic Syndrome / 대한소화기학회지

Endoscopic submucosal dissection (ESD) has been successfully performed in thrombocytopenic conditions such as in patients with liver cirrhosis but successful ESD for early gastric cancer (EGC) in hematologic diseases has rarely been reported. A 52-year-old male patient, who had previously been diagnosed with myelodysplastic syndrome 2 years ago, was admitted to our hospital for ESD of EGC. ESD was performed successfully in this patient after platelet concentrates transfusion on the day of ESD. ESD might be an option for the treatment of EGC in thrombocytopenia due to hematologic diseases when optimal supportive managements are applied.

Successful treatment of myelodysplastic syndrome and Behcet colitis after allogeneic hematopoietic stem cell transplantation

No abstract available.

Infecção fulminante por Clostridium septicum: relato de caso / Fulminant Clostridium septicum infection: case report

As infecções de tecidos moles causadas por espécies de Clostridium têm sido descritos na literatura por centenas de anos. A gangrena gasosa por Clostridium continua sendo uma importante causa de morbidade e mortalidade no mundo. O objetivo deste estudo foi relatar um caso de paciente com diagnóstico de síndrome mielodisplásica, submetida à curetagem uterina e evoluindo com gangrena gasosa espontânea, 10 horas após a cirurgia.Paciente do sexo feminino, 26 anos, com história de dor em terço distal de membros inferiores, irradiando para região de fossa poplítea, com piora à palpação e movimentação dos membros, acompanhada de aumento da temperatura e volume local. Negava febre, hiperemia ou trauma local, evoluindo para choque séptico. Angiotomografia das extremidades e pelve revelou a presença de gás permeando os feixes musculares da coxa e da perna, bilateralmente. A combinação da história e exame clínico,ao estudo radiológico confirmou o diagnóstico sindrômico de gangrena gasosa espontânea. Apesar de elevado índice de suspeição melhorar os resultados clínicos, tais infecções progridem tão rapidamente que o óbito pode preceder o diagnóstico,não obstante, o reconhecimento precoce e tratamento agressivo,incluindo drenagem aberta ou percutânea. Antibióticos parenterais contra Clostrídios devem ser prontamente iniciados, bem como medidas de suporte clínico.

The soft tissue infections caused by Clostridium species have been described in the literature for hundreds of years. The gas gangrene due to Clostridium remains an important cause of morbidity and mortality worldwide. The aim of this study was to report a patient diagnosed with myelodysplastic syndrome who underwent curettage, evolving with spontaneous gas gangrene, 10 hours after surgery. Female patient, 26 years, with pain in the distal third of the lower limbs, radiating to the popliteal fossa region, which worsened on palpation and movement of limbs, and accompanied by an increase in temperature and local volume. She denied fever, redness or local trauma, and progressed to septic shock. Angiotomography of the extremities and pelvis revealed the presence of gas permeating the muscle bundles of the thigh and leg bilaterally. The combination of history and clinical examination and radiological examination confirmed the syndromic diagnosis of Spontaneous Gas Gangrene. Although a high index of suspicion may improve clinical outcomes, such infections progress so rapidly that death usually precedes the diagnosis. However, early recognition and aggressive treatment, including open or percutaneous drainage and parenteral antibiotics against Clostridia should be promptly initiated, along with clinical support.

A Case of Inguinal Sparganosis Mimicking Myeloid Sarcoma

We report here a case of inguinal sparganosis, initially regarded as myeloid sarcoma, diagnosed in a patient undergone allogeneic hematopoietic transplantation (HSCT). A 56-year-old male patient having myelodysplastic syndrome was treated with allogeneic HSCT after myeloablative conditioning regimen. At day 5 post-HSCT, the patient complained of a painless palpable mass on the left scrotum and inguinal area. Pelvic magnetic resonance imaging and computed tomography revealed suspected myeloid sarcoma. Gun-biopsy was performed, and the result revealed eosinophilic infiltrations without malignancy. Subsequent serologic IgG antibody test was positive for sparganum. Excisional biopsy as a therapeutic diagnosis was done, and the diagnosis of sparganosis was confirmed eventually. This is the first report of sparganosis after allogeneic HSCT mimicking myeloid sarcoma, giving a lesson that the physicians have to consider the possibility of sparganosis in this clinical situation and perform adequate diagnostic and therapeutic approaches.

Detection of RUNX1-MECOM Fusion Gene and t(3;21) in a Very Elderly Patient Having Acute Myeloid Leukemia with Myelodysplasia-Related Changes

An 87-yr-old woman was diagnosed with AML with myelodysplasia-related changes (AML-MRC). The initial complete blood count showed Hb level of 5.9 g/dL, platelet counts of 27x10(9)/L, and white blood cell counts of 85.33x10(9)/L with 55% blasts. Peripheral blood samples were used in all the tests, as bone marrow examination could not be performed because of the patient's extremely advanced age and poor general health condition. Flow cytometric analysis, chromosome analysis, FISH, and reverse transcriptase-PCR (RT-PCR) results indicated AML-MRC resulting from t(3;21) with the RUNX1-MECOM fusion gene. To our knowledge, this is the second most elderly de novo AML patient associated with t(3;21) to be reported.

Síndrome de Sweet e policondrite recidivante reveladores de síndrome mielodisplásica / Sweet's Syndrome and relapsing polychondritis signal myelodysplastic syndrome

Certas dermatoses, pertencentes ao grupo das síndromes paraneoplásicas mucocutâneas, podem ser o prenúncio de uma neoplasia previamente não conhecida. Tanto a síndrome de Sweet como a policondrite recidivante incluem-se neste grupo. A síndrome de Sweet e a PR são raramente encontradas em um mesmo paciente. A presença de policondrite recidivante e síndrome de Sweet em um mesmo paciente tem se revelado mais frequente em pacientes com neoplasias associadas, sobretudo hematológicas. Relata-se o caso de paciente do sexo masculino, 79 anos, com síndrome de Sweet e policondrite recidivante, em quem, subsequentemente, foi diagnosticada uma síndrome mielodisplásica.

The emergence of certain skin conditions belonging to the group of mucocutaneous paraneoplastic syndromes may indicate the future appearance of a previously unknown malignancy. Sweet's Syndrome and relapsing polychondritis are included in this group. Sweet's Syndrome and relapsing polychondritis are very rarely found together in the same patient. This dual occurrence is more commonly found in cancer patients with associated hematological malignancies. We report the case of a 79year-old male with Sweet's Syndrome and relapsing polychondritis, who was subsequently diagnosed with a myelodysplastic syndrome.

Trombosis venosa portal extrahepática asociada a infección por citomegalovirus en una paciente con síndrome mielodisplásico: reporte de un caso y revisión de la literatura / Extrahepatic portal vein thrombosis associated with cytomegalovirus infection in a female patient with myelodysplastic syndrome: report of a case and review of literature

La trombosis venosa portal extrahepática (TVPHE) ocurre en etapas tempranas de la vida como complicación de onfalitis, canulación de vena umbilical, sepsis intraabdominal, deshidratación y estados de hipercoagulabilidad. En la adultez, se describe la asociación a estados de hipercoagulabilidad, principalmente deficiencias de proteínas C y S, postraumática, manipulación portal, pancreatitis, así como obstrucción o invasión tumoral. En la mitad de estos casos, la etiología es desconocida. Hay reportes en la literatura de TVPEH asociada a procesos infecciosos severos como sepsis por Fusobacterium necrophorum, Fusobacterium nucleatum, así como infección por Citomegalovirus; sin embargo estos reportes son muy escasos. Paciente femenino de 56 años de edad, con antecedente de síndrome mielodisplásico, quien acude por clínica de dolor abdominal difuso, ictericia obstructiva y trastornos en el funcionalismo hepático. Dentro de las evaluaciones efectuadas se realiza Ultrasonido Endoscópico (USE) con evidencia de trombosis venosa profunda portal y mesentérica superior con hipertensión portal, várices esofágicas y fundicas. Se plantea probable origen infeccioso por lo que se solicita serologia para CMV con niveles elevados de IgG, se inicia tratamiento antiviral con valganciclovir y betabloquenates con evolución satisfactoria luego de un año. Conclusión: A pesar de que la trombosis venosa portal asociada a la infección por CMV es un evento poco frecuente en individuos inmunocompetentes, el descarte de esta debe ser incluida dentro del plan diagnóstico de trombosis del lecho esplenoportal...

Extrahepatic portal vein thrombosis (EHPVT) occurs in early stages of life as a complication of omphalitis, cannulation of the umbilical vein, intra-abdominal sepsis, dehydration, and hypercoagulable states, mainly C and S proteins deficiencies, post-traumatic, portal vein manipulation, pancreatitis, as well as tumor invasion or obstruction. Etiology is unknown in half of these cases. In the literature, there are reports of EHPVT associated with severe infectious processes such as sepsis by Fusobacterium necrophorum, Fusobacterium nucleatum, as well as infection by Cytomegalovirus (CMV). Nonetheless, such reports are very scarce. Female patient aged 56, con antecedent myelodysplastic syndrome, who attends the clinic with diffuse abdominal pain, obstructive icterus, and liver function disturbances. Among the assessments performed, an Endoscopic Ultrasound (EUS) is carried out evidencing upper mesenteric and deep portal vein thrombosis with portal hypertension, esophageal and fundic varices. A probable infectious origin is stated, thus serology is requested for CMV with elevated levels of IgG; antiviral treatment is started with Valgancyclovir and Beta-Blockers with satisfactory evolution after one year. Conclusion: Despite the portal vein thrombosis associated with the infection by CMV is a rare event in immunocompetetnt persons, discarding it must be included in a diagnosis plan for splenic-portal bed thrombosis...

Urolithiasis in Patients Suffering from Malignant Hematologic Diseases

PURPOSE: We performed this study in order to evaluate the incidence and characteristics of urolithiasis in patients with malignant hematologic diseases. MATERIALS AND METHODS: Nine hundred one patients who underwent medical treatment for malignant hematologic disease and 40,543 patients who visited the emergency room and without malignant hematologic diseases were included in our study. The patients with malignant hematologic diseases were divided into two groups depending on their primary treatment. Group I included patients with acute and chronic leukemia (AML, ALL, CML, CLL) for which chemotherapy and steroid therapy was necessary, and group II included patients with anaplastic anemia and myelodysplastic syndrome and who had undergone repeated transfusion for treatment. Comparisons were made between the two groups in respect to the incidence of urolithiasis and the stones' radiopacity. RESULTS: Twenty nine patients (3.2%) of the 901 malignant hematologic patients were diagnosed with urolithiasis, compared to 575 patients (1.4%) of 40,543 emergency room patients. There was a significant increase of the incidence of urolithiasis in the malignant hematologic group. Compared to the general patients, the patients with malignant hematologic diseases had a higher rate of radiolucent stones (46.6% versus 16.3%, respectively), and the difference was significant. CONCLUSION: The incidence of urolithiasis for malignant hematologic patients was significantly higher than that for the control group.

Clinical Importance of Morphological Multilineage Dysplasia in Acute Myeloid Leukemia with Myelodysplasia Related Changes / 대한진단검사의학회지

BACKGROUND: AML with myelodysplasia related changes (AML MRC) is known to show a poor prognosis compared with de novo AML, but controversies exist about the prognostic impact of multilineage dysplasia (MLD) among MRC. We investigated the prognostic impact of MLD in AML MRC. METHODS: A total of 357 patients newly diagnosed as AML at Asan Medical Center from January 2001 to December 2005 were analyzed. They were diagnosed and classified as AML with recurrent genetic abnormalities, AML MRC, and AML not otherwise specified (AML NOS). Prognostic markers including overall survival (OS) and event free survival (EFS) were obtained through retrospective analysis of electronic medical records. RESULTS: AML MRC patients showed a lower complete remission (CR) rate (44.7% vs. 64.9%, P=0.002) and shorter OS (297 vs. 561 days, P=0.004) and EFS (229 vs. 374 days, P=0.004) than AML NOS patients. Patients with MLD among AML MRC also showed a lower CR rate (37.7%, P=0.001) and shorter OS (351 days, P=0.036) and EFS (242 days, P=0.076) than AML NOS patients. However, among AML MRC patients, there were no differences in OS, EFS and CR between patients with and without MLD. CONCLUSIONS: AML MRC patients showed a lower CR rate and shorter OS and EFS than AML NOS patients. AML MRC patients with MLD showed similar results and their prognosis was not different from those without MLD. MLD findings among AML MRC could be an independent poor prognostic factor in de novo AML.

Peliosis hepatis como complicación del uso de anticonceptivos orales en una paciente con mielodisplasia / Peliosis hepatis as a complication of the ise of oral contraceptives in a patient with myelodysplasia

Introduction: Peliosis hepatis (PH) is an uncommon condition in pediatrics; however, it is one of the most serious complications associated with the long-term use of use of steroids. It is characterized by multiple blood-filled cavities, mostly involving the liver. Myelodysplastic Syndrome (MDS) is also a complex and infrequent hematological condition; it may transform into acute leukemia and its treatment requires medications that may lead to PH. Case Report: 13 year-old girl with MDS, refractory cytopenia type. A family donor for SCL was not available, therefore immunosuppressive treatment, steroids and transfusions were initiated. Due to metrorrhage, estrogen was used at high doses. She developed acute abdominal pain; abdominal ultrasound and CL scan showed PH and peritoneal bleeding. Oral contraceptives were decreased resulting in reduction of PH, but a new episode of uterine bleeding causing hypovolemic shock forced a hysterectomy in order to suspend estrogen treatment. Due to lack of response to treatment to SMD, she continued been treated with transfusions as needed, and died 32 months post diagnosis. Discussion: PH is an uncommon and life-threatening condition in children receiving prolonged treatment with steroids. Current modalities of SCL in patients with MDS will replace the need for steroids, thus avoiding this severe complication.

Introducción: La Peliosis Hepática (PH) es una condición muy infrecuente en pediatría, caracterizada por la presencia de múltiples cavidades sanguíneas en el parénquima hepático, asociada al uso prolongado de estrógenos o corticoides, El Síndrome Mielodisplásico (SMD) es una alteración hematológica compleja que puede evolucionar a leucemia y que puede requerir para su tratamiento medicamentos relacionados al desarrollo de PH. Caso Clínico: Niña 13 años, con SMD tipo citopenia refractaria, con dependencia transfusional, sin posibilidad de realizar Trasplante de progenitores hematopoyéticos (TPH) por falta de donante familiar compatible. Recibió transfusiones, inmunosupresores y corticoides por tiempo prolongado. Presentó metrorragias severas requiriendo estrógenos en altas dosis. Evolucionó con hemoperitoneo, diagnosticándose PH por ecografía y scanner abdominal. Al reducir dosis de estrógenos disminuyeron lesiones hepáticas, pero nuevo episodio de metrorragia con shock hipovolémico, obligó a realizar histerectomía para suspender estrógenos. Sin respuesta a tratamiento del SMD, se mantuvo con transfusiones según requerimiento y falleció a los 32 meses del diagnóstico. Discusión: La PH es una complicación grave, que podría evitarse con el desarrollo de nuevas técnicas de TPH que permiten contar con donantes no relacionados para el tratamiento de síndromes de falla medular como el SMD.