Aplasia medular congénita de serie roja: Anemia de DiamondBlackfan. A propósito de un caso / Marrow aplasia congenital red series: Diamond BlackfanAnemia. About a case

La Aplasia Medular pura de Células Rojas es un trastorno que se caracteriza por anemia con ausencia casi completa de precursores de células rojas en la médula ósea, con contaje de leucocitos y plaquetas normales. La anemia de Diamond-Blackfan es un síndrome de insuficiencia de la médula ósea caracterizada por anemia, reticulocitopenia y disminución de precursores eritroides en la médula ósea. Se presenta el caso de un lactante menor masculino de 2 meses de edad, sin antecedentes familiares ni perinatales de importancia, cuya madre evidencia palidez cutáneo mucosa progresiva asociado a hiporexia; acude a centro de salud donde realizan paraclínicos que reportan hemoglobina en 1,7g/dL. Se realiza frotis de sangre periférica donde se muestra serie eritroide francamente afectada con contaje granulocítico y megacariocítico normales; se realiza biopsia y aspirado de médula ósea concluyéndose aplasia medular de serie roja y en vista de otros hallazgos clínicos, se plantea posible anemia de Diamond-lackfan. Se indica tratamiento con glucocorticoides, sin embargo por respuesta insuficiente, se inicia eritropoyetina aumentando dosis de forma progresiva, a pesar de la administración de la misma, amerita transfusiones sanguíneas de forma regular; se realizan estudios de compatibilidad con familiares de primer grado resultando positivos, actualmente es candidato a trasplante alogénico de médula ósea. Se concluye que a pesar de corresponder a un síndrome poco frecuente, debe sospecharse ante la presencia de anemia severa, sin pérdida sanguínea aguda y descarte previo de otras etiologías, además se plantea que el inicio oportuno del tratamiento es fundamental para la supervivencia de estos pacientes(AU)

Pure red cell aplasia medullary is a disorder characterized by anemia with almost complete absence of red cell precursors in the bone marrow, with leukocyte count and platelets. e Diamond-Blackfan anemia is a failure syndrome characterized by bone marrow anemia, reticulocytopenia and decreased erythroid precursors in the bone marrow. the case of an infant under 2 months of age presented no family or perinatal history major, whose mother progressive skin pallor evidence mucosa associated with hyporexia; go to health center where they perform paraclinical reporting hemoglobin 1.7 g /dL. peripheral blood smear where erythroid frankly affected with normal megakaryocytic granulocytic count shown is made; It biopsied and bone marrow aspirate concluding marrow red cell aplasia; possible anemia Diamond-Blackfan in light of other clinical findings arises. It stays with glucocorticoid treatment, however insufficient response, begins erythropoietin dose progressively increasing, despite it, warrants blood transfusions on a regular basis; compatibility studies performed with firstdegree resulting positive, currently a candidate for allogeneic bone marrow transplantation. It is concluded that despite being a rare syndrome should be suspected in severe anemia where there is acute blood loss, ruling out other etiologies; also timely initiation of treatment is critical to the survival of these patients(AU)

Aplasia pura adquirida crônica da série vermelha secundária à quimioterapia em paciente com adenocarcinoma endometrioide invasivo / Chronic acquired pure aplasia of the red series secondary to chemotherapy in a patient with invasive endometrioid adenocarcinoma

O presente relato de caso descreve a apresentação atípica de uma paciente com adenocarcinoma endometrioide invasivo que evoluiu com aplasia pura adquirida crônica da série vermelha secundária à quimioterapia. Paciente de 71 anos, sexo feminino, procurou atendimento médico por quadro de metrorragia com três meses de evolução. A curetagem uterina evidenciou adenocarcinoma endometrioide invasor moderadamente diferenciado. Iniciou-se uma abordagem com esquema quimioterápico composto por Carboplatina e Paclitaxel interrompido ao quinto ciclo para evitar progressão de aplasia medular constatada por biópsia de medula óssea. A possível hematotoxicidade do protocolo Paclitaxel e Carboplatina foi observada na conduta terapêutica da paciente, por sua progressão para uma apresentação atípica de aplasia pura adquirida crônica da série vermelha após administração desta associação de drogas.

The present case report describes the atypical presentation of a patient with invasive endometrioid adenocarcinoma that evolved with chronic acquired pure aplasia of the red series secondary to chemotherapy. A seventy-one-yearold patient, female, sought medical care for a three-month-old metrorrhagia evolution. The uterine curettage showed moderately differentiated invasive endometrioid adenocarcinoma. It was initiated an approach with chemotherapy regimen consisting of Carboplatin and Paclitaxel interrupted at the fifth cycle to prevent progression of spinal aplasia found by bone marrow biopsy. The possible hematoxicity of the patient, for its progression to an atypical presentation of chronic acquired aplasia of the red series after administration of this combination of drugs.

Pure red-cell aplasia secondary to pregnancy: Case report and review of the literature / Aplasia eritroide pura secundaria a embarazo: reporte de un caso y revisión de la literatura

RESUMEN Objetivo: Reportar el caso de una aplasia eritroide pura secundaria al embarazo y hacer una revisión de la literatura sobre el diagnóstico, tratamiento y pronóstico materno y perinatal. Materiales y métodos: Se presenta el caso de una paciente de 24 años, con embarazo de 34 semanas, remitida a un hospital público de referencia regional por anemia en estudio; se realiza biopsia de médula ósea sobre la que se hace diagnóstico de aplasia eritroide pura asociada al embarazo. Recibe manejo con transfusiones seriadas de glóbulos rojos; se realiza cesárea a término, con recién nacido sano; en su puerperio presenta estabilidad de los valores de hemoglobina. Se realizó búsqueda bibliográfica en las bases de datos electrónicas de Medline vía PubMed, LILACS, SciELO y ScienceDirect, con los términos "embarazo", "aplasia pura de células rojas", "pregnancy" y "pure red-cell aplasia". Se incluyeron reportes de caso, series de casos y revisiones bibliográficas en inglés y español, desde enero de 1999 a enero de 2020, de pacientes gestantes con aplasia eritriode pura. Se tomó información sobre el diagnóstico, tratamiento y pronóstico materno y perinatal. Tres autores seleccionaron los estudios por título y resumen. Se hace síntesis descriptiva. Resultados: se identificaron 828 títulos, de los cuales 818 fueron descartados luego de revisar los criterios de inclusión. Se incluyeron diez artículos: seis reportes de casos, tres reportes de caso con revisión de la literatura, un reporte de caso en la modalidad de poster, para un total de 10 casos re- portados. El diagnóstico se basó en bajos niveles de hemoglobina y compromiso de la línea eritriode en la biopsia de médula. El tratamiento en transfusiones de glóbulos rojos y el pronóstico materno-fetal fueron buenos. Conclusión: el diagnóstico de la aplasia eritroide pura en el embarazo requiere biopsia de médula. El pronóstico materno-perinatal es bueno, con soporte transfusional. Se requieren más estudios para evaluar la seguridad y eficacia de los corticosteroides para esta entidad en el embarazo.

ABSTRACT Objective: To report a case of pure red-cell aplasia secondary to pregnancy and to conduct a review of the literature regarding diagnosis and treatment, as well as maternal and perinatal prognosis. Materials and methods: This is the case of a 24-year-old patient at 34 weeks of gestation, referred to a regional public referral hospital due to anemia. Bone marrow biopsy was performed, leading to the diagnosis of pregnancy-related pure red-cell aplasia. The patient received serial red blood cell transfusions. Delivery by Cesarean section at term resulted in a healthy newborn. Hemoglobin values remained stable during the postoperative period. A literature search was conducted in Medline via PubMed, LILACS, SciELO and ScienceDirect using the terms "pregnancy" and "pure red-cell aplasia". Case reports, case series and literature reviews in English and Spanish published between January 1999 and January 2020 that report pregnant women with pure red-cell aplasia were included. Information on diagnosis, treatment and maternal and perinatal prognosis was collected. Three of the authors selected the studies by title and abstract; A descriptive synthesis is provided. Results: Overall, 828 titles were identified; of these,818 were discarded after reviewing the inclusions criteria. Ten articles were included: six case reports, three case reports with literature review, and one case report in the poster modality, for a total number of 10 reported cases. Diagnosis was based on low hemoglobin levels and compromised erythroid cell line in bone marrow biopsy. Treatment consists of red blood cell transfusions, with good maternal and fetal prognosis. Conclusion: Diagnosis of pure red-cell aplasia during pregnancy requires bone marrow biopsy. With transfusion support, maternal perinatal prognosis is good. Further studies are required to assess the safety and efficacy of steroid use in this pregnancy-related condition.

Pure red cell aplasia and anti-erythropoietin antibodies in patients on hemodialysis: a report of two cases and a literature review / Aplasia pura de células vermelhas e anticorpo antieritropoietina em pacientes de hemodiálise: relato de dois casos e revisão da literatura

ABSTRACT Introduction: Anemia is a frequent multifactorial complication of CKD seen in patients on dialysis derived mainly from impaired erythropoietin (EPO) production. A less common cause of anemia in individuals with CKD is pure red cell aplasia (PRCA) secondary to the production of anti-EPO antibodies. Objective: This paper aimed two describe two cases of PRCA secondary to the production of anti-EPO antibodies including choice of treatment, patient progression, and a literature review. Materials: This study included the cases of two patients with CKD on hemodialysis with severe anemia in need of specific investigation and management. Results: Patient 1 with CKD secondary to hypertension treated with EPO for 7 months showed persistent decreases in hemoglobin (Hb) levels despite the subcutaneous administration of increasing doses of EPO; the patient required recurring blood transfusions. Workup and imaging tests were negative for the main causes of anemia in individuals with CKD on dialysis. Patient 2 with CKD secondary to adult polycystic kidney disease had been taking EPO for 2 years. The patient developed severe abrupt anemia the month he was started on HD, and required recurring transfusions to treat the symptoms of anemia. Workup and imaging findings were inconclusive. Specific laboratory tests confirmed the patients had anti-EPO antibodies. After six months of immunosuppressant therapy (corticosteroids + cyclosporine) the patients were stable with Hb > 9.0 g/dl. Conclusion: PRCA is a rare condition among patients on dialysis treated with rhEPO and should be considered as a possible cause of refractory anemia. Treating patients with PRCA may be challenging, since the specific management and diagnostic procedures needed in this condition are not always readily available.

RESUMO Introdução: Anemia é complicação frequente da Doença Renal Crônica (DRC) em pacientes dialíticos. Apresenta caráter multifatorial principalmente pela insuficiente produção de eritropoietina (EPO). Situação rara causadora de anemia na DRC é Aplasia Pura de Células Vermelhas (APCV), em decorrência da produção de anticorpos anti-EPO. Objetivo: Descrever 2 casos de APCV com formação de anticorpos anti-EPO, sua abordagem clínica, evolução e revisão de literatura. Métodos: Dois pacientes em hemodiálise que desenvolveram anemia grave, necessitando investigação e manejo específico. Resultados: Paciente nº 1: feminina, 75 anos, DRC secundária à hipertensão arterial. Após 7 meses com EPO desenvolveu queda persistente em valores de hemoglobina (Hb) mesmo com incremento em doses EPO SC, necessitando transfusões de sangue recorrentes. Extensa investigação laboratorial e de imagem resultou negativa para principais causas de anemia. Paciente nº 2: masculino, 66 anos, DRC secundária à DRPA, há 2 anos em uso de EPO. No mês de entrada em HD desenvolveu anemia severa, também exigindo transfusões recorrentes para tratamento da anemia sintomática. Extensa investigação laboratorial e por imagem, sem chegar a uma conclusão definitiva. Em ambos os casos a presença de anticorpos anti-EPO foi confirmada por exames laboratoriais específicos. Terapia imunossupressora resultou em estabilização do quadro e Hb > 9,0 g/dl em ambos os pacientes, 6 meses após início do tratamento. Conclusão: APCV é condição rara entre pacientes dialíticos que recebem EPOHuR e deve ser lembrada como causa de anemia refratária. Seu manejo específico e diagnóstico laboratorial nem sempre acessível, tornando desafiadora a condução dos casos para o nefrologista.

Pure red cell aplasia in children: a clinical analysis of 16 cases / 中国当代儿科杂志

OBJECTIVE@#To study the clinical features, treatment, and prognosis of pure red cell aplasia (PRCA) in children.@*METHODS@#A retrospective analysis was performed for the clinical data of 16 children with PRCA. The outcome and prognosis of patients treated with prednisone combined with Huaiqihuang granules versus prednisone alone were evaluated.@*RESULTS@#All the 16 children complained of symptoms of anemia including pale or sallow complexion. Of 12 children undergoing pathogen test, 7 (58%) were found to have pathogen infection, among which human cytomegalovirus was the most common. Lymphocyte subsets were measured for 7 children, among whom 5 (71%) had lymphocyte immune disorder. Six children were found to have abnormalities in immunoglobulin and complement. The 8 children treated with prednisone combined with Huaiqihuang granules had a median follow-up time of 21.5 months, among whom 1 was almost cured, 1 was relieved, and 6 were obviously improved; the median onset time of treatment was 1 month, and 2 children had disease recurrence in the course of drug reduction or withdrawal. The 8 children in the prednisone alone treatment group had a median follow-up time of 34 months, among whom 4 were almost cured, and 4 were obviously improved; the median onset time of treatment was 2.5 months, and 4 children had recurrence during drug reduction or withdrawal.@*CONCLUSIONS@#Children with PRCA usually complain of anemia-related symptoms. Laboratory tests show pathogen infection in some children with PRCA, and most of children have immune disorders. Glucocorticoids have a good therapeutic effect, but some children relapse in the course of drug reduction or withdrawal. Combined treatment with prednisone and Huaiqihuang granules may have a faster onset of action and less possibility of recurrence.

Aplasia pura de células rojas secundaria a infección por parvovirus B19 en paciente VIH positivo / Pure red cell aplasia secondary to parvovirus B19 infection in an HIV positive patient

Resumen La infección crónica por parvovirus B19 en pacientes seropositivos para el virus de inmunodeficiencia humana (VIH) es una causa identificable y tratable de anemia en esta población, que en nuestro medio aún no ha sido muy estudiada. La búsqueda activa de este patógeno en pacientes VIH positivos con anemia grave sin compromiso de otras líneas celulares puede llevar a una mejoría en la calidad de vida en el tiempo y reducción de los costos de la enfermedad para el sistema de salud. Nosotros presentamos el caso de un paciente con síndrome de inmunodeficiencia adquirida y anemia a quien se le identifico parvovirus B19. (Acta Med Colomb 2018; 43: 42-44).

Abstract The chronic infection by parvovirus B19 in seropositive patients for the human immunodeficiency virus (HIV) is an identifiable and treatable cause of anemia in this population, which in our environment has not yet been widely studied. The active search for this pathogen in HIV positive patients with severe anemia without compromising other cell lines can lead to an improvement in the quality of life over time and reduction of the costs of the disease to the health system. We present the case of a patient with acquired immunodeficiency syndrome and anemia who was identified as parvovirus B19. (Acta Med Colomb 2018; 43: 42-44).

Del(5q) myelodysplastic syndrome combined with pure red cell aplasia

No abstract available.

Pure Red Cell Aplasia Associated with Good Syndrome

Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive therapy, and provide a review of the pertinent literature.

Significance of clonal TCR gene rearrangement in acquired pure red cell aplastic anemia / 中华医学遗传学杂志

<p><b>OBJECTIVE</b>To evaluate the significance of T-cell antigen receptor (TCR) gene rearrangement among patients with acquired pure red cell aplastic anemia (A-PRCA).</p><p><b>METHODS</b>For 16 patients with A-PRCA, an immunosuppressive regimen based on cyclosporin A (CsA) was applied. Rearrangement of the TCR gene was detected by PCR, and T lymphocyte subsets in peripheral blood specimens was detected with flow cytometry.</p><p><b>RESULTS</b>Five patients had presented with TCR clonal rearrangement and were positive for both TCR γ and TCR δ. The blood of 13 patients have returned to normal with the treatment, which included 3 cases with bone marrow returning to normal. In 7 cases, the red cell hyperplasia of bone marrow is still down, but has increased with the treatment. Three patients were close to cure, 7 showed remission, 3 were improved, but 3 were ineffective. The rate of effective treatment in those with TCR rearrangement (2/5) was significantly lower than that those without (11/11, chi-square=8.123, P < 0.05). Compared with those without the TCR gene rearrangement, the Th cells and proportion of Th/Ts were significantly lower, while the Ts cell (CD3+CD8+) were significantly higher in those with the rearrangement (P < 0.05).</p><p><b>CONCLUSION</b>TCR gene rearrangement may play a role in the pathogenesis of A-PRCA. CsA is effective for the treatment of A-PRCA, but patients presenting clonal TCR gene rearrangement may response poorly to the treatment.</p>

Primary acquired chronic pure red cell aplasia refractory to standard treatments: remission with rituximab

No abstract available.

Safety and Efficacy of Intravitreal Ganciclovir Injections More than 10 Times for Cytomegalovirus Retinitis

PURPOSE: To study the treatment outcomes in patients who were administered multiple intravitreal ganciclovir injections more than 10 times alone without systemic anti-cytomegalovirus therapy for cytomegalovirus retinitis. CASE SUMMARY: A 64-year-old man who underwent immunosuppressive therapy after thymectomy due to an invasive thymoma and pure red-cell aplasia, a 60-year-old woman who underwent chemotherapy after diagnosis of diffuse large B-cell lymphoma, a 49-year-old man with a history of bone marrow transplantation due to acute myeloid leukemia, a 29-year-old woman with dermatomyositis treated with oral steroids and cyclosporine, and a 47-year-old woman who received intravitreal dexamethasone implant injections, intravitreal and subtenon steroid injections due to Behcet's disease were diagnosed with cytomegalovirus retinitis. All patients showed systemic complications such as pancytopenia after systemic anti-cytomegalovirus therapy, and therefore, they were administered multiple intravitreal ganciclovir injections alone. Best-corrected visual acuities improved in all patients, except in one case, where viral lesions were observed in the fovea. Retinal hemorrhaging and infiltrative lesions decreased in all patients. No severe complication was observed during the injection and in the follow-up period. CONCLUSIONS: Multiple intravitreal ganciclovir injections alone can be used as a treatment modality for cytomegalovirus retinitis to avoid the systemic side effects of systemic anti-cytomegalovirus therapy.

Acquired Pure Red Cell Aplasia following Autoimmune Hemolytic Anemia in Systemic Lupus Erythematosus / 대한내과학회지

Pure red cell aplasia (PRCA) is a rare hematological disorder characterized by severe normochromic normocytic anemia and reticulocytopenia due to erythroid progenitor depletion in an otherwise normal bone marrow. Autoimmune hemolytic anemia (AIHA) is caused by autoantibodies directed against red blood cells with normocytic or macrocytic anemia with reticulocytosis. Both diseases can develop in conjunction with various underlying diseases, such as immunological disorders. Although rare, there have been a few cases of AIHA followed by PRCA. Here, we report a patient who developed PRCA following AIHA and was later diagnosed with systemic lupus erythematosus.

Impacts of ABO incompatibility on early outcome after single unit unrelated cord blood transplantation: a retrospective single center experience / 中华血液学杂志

<p><b>OBJECTIVE</b>To retrospectively study the impacts of ABO incompatibility on early outcome after single unit unrelated cord blood transplantation（UCBT）, such as cumulative incidence of engraftment, incidence of acute graft- versus- host disease （aGVHD） and 180- day transplant- related mortality（TRM）.</p><p><b>METHODS</b>208 patients underwent single unit UCBT from April 2008 to October 2014 were analyzed, included 99 ABO- identical, 60 minor, 38 major and 11 bidirectional ABO- incompatible recipients. All the patients received intensified myeloablative conditioning, and a combination of cyclosporine A and mycophenolate mofetil was given for GVHD prophylaxis.</p><p><b>RESULTS</b>Cumulative incidences of neutrophil engraftment, platelet recovery, erythroid lineage reconstitution, Ⅱ-Ⅳ aGVHD, Ⅲ-Ⅳ aGVHD and 180- day TRM showed no significant difference among the patients receiving ABOidentical, minor, major, and bidirectional UCBT（all P>0.05, respectively）. What's more, none of the patients developed pure red- cell aplasia（PRCA）after UCBT. Group A donor and a group O recipient patients didn't appeared to influence the clinical results when compared with others（all P>0.05, respectively）.</p><p><b>CONCLUSION</b>Patients receive ABO- incompatible UCBT may not develop PRCA. The presence of ABO- incompatibility did not influence the hematopoietic reconstitution, the incidence of aGVHD and 180-day TRM in this cohort. There is not support for the need to regard ABO-compatibility as an UCB-graft selection criterion.</p>

Spontaneous Remission in a Teenage Girl with Acquired Pure Red Cell Aplasia / 임상소아혈액종양

Acquired pure red cell aplasia (PRCA) can be induced by various factors such as viral infection, thymoma, connective tissue disease, lymphoma, and adverse drug reactions. PRCA has not been reported in an adolescent in Korea for the past several decades. We recently experienced a case of acquired PRCA in an adolescent. A 14-year-old girl presented with pallor, dizziness, and mild fever. She had isolated normocytic normochromic anemia with reticulocytopenia in the peripheral blood and erythroblastopenia in the bone marrow. She was diagnosed with secondary acquired PRCA presumably induced by Mycoplasma pneumoniae infection during her clinical course, and she experienced spontaneous remission 11 weeks after initial diagnosis. Her clinical and hematologic statuses were normal as far as 20 months after her diagnosis.

Spontaneous Remission in a Teenage Girl with Acquired Pure Red Cell Aplasia / 임상소아혈액종양

Acquired pure red cell aplasia (PRCA) can be induced by various factors such as viral infection, thymoma, connective tissue disease, lymphoma, and adverse drug reactions. PRCA has not been reported in an adolescent in Korea for the past several decades. We recently experienced a case of acquired PRCA in an adolescent. A 14-year-old girl presented with pallor, dizziness, and mild fever. She had isolated normocytic normochromic anemia with reticulocytopenia in the peripheral blood and erythroblastopenia in the bone marrow. She was diagnosed with secondary acquired PRCA presumably induced by Mycoplasma pneumoniae infection during her clinical course, and she experienced spontaneous remission 11 weeks after initial diagnosis. Her clinical and hematologic statuses were normal as far as 20 months after her diagnosis.

Pure red-cell aplasia and autoimmune hemolytic anemia in a patient with acute hepatitis A

Pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) have rarely been reported as an extrahepatic manifestation of acute hepatitis A (AHA). We report herein a case of AHA complicated by both PRCA and AIHA. A 49-year-old female with a diagnosis of AHA presented with severe anemia (hemoglobin level, 6.9 g/dL) during her clinical course. A diagnostic workup revealed AIHA and PRCA as the cause of the anemia. The patient was treated with an initial transfusion and corticosteroid therapy. Her anemia and liver function test were completely recovered by 9 months after the initial presentation. We review the clinical features and therapeutic strategies for this rare case of extrahepatic manifestation of AHA.

A case of bilateral tibial hemimelia type VIIa.

Congenital absence of tibia is a rare anomaly, and may be total or partial, unilateral or bilateral. Total absence is more frequent than partial, unilateral absence occurs more often than bilateral, with right limb more commonly affected than the left. In partial defect, almost always the distal end of the bone is affected, and of the bilateral cases, there may be total absence on both sides, or total on one side and partial on the other. Males are slightly more commonly affected than the females. Though, the family history is usually negative for congenital abnormalities and other diseases, there is a considerable chance of occurrence of congenital defect of the tibia or of other abnormalities, in near or remote relatives. We report a case of newborn having bilateral tibial hemimelia type VIIa.

Pure red cell aplasia with thymoma/T-cell large granular lymphocyte leukemia: two cases report and literatures review / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical and laboratory features of 2 cases of pure red cell aplasia (PRCA) with thymoma/T-cell large granular lymphocyte leukemia (T-LGLL), and to improve the recognition of the disease and the role of lymphocyte in its mechanism.</p><p><b>METHODS</b>Two cases of PRCA with thymoma/T-LGLL were reported and the related literatures were reviewed.</p><p><b>RESULTS</b>Case 1 was a 63-years old male with hemoglobin level of 54 g/L at admission. Case 2 was a 52-years old female with hemoglobin level of 79 g/L at admission. They were both diagnosed as PRCA with thymoma before admission to our hospital and had no benefit from their thymectomy. Further examinations in our hospital showed that CD3⁺CD4⁻CD8⁺CD57⁺ large granular lymphocytes amplified with clonal TCR rearrangement in their peripheral blood. The diagnosis of PRCA with thymoma/T-LGLL was clarified. Case 1 did not respond to any of the frontline therapies while case 2 responded completely to cyclosporine.</p><p><b>CONCLUSION</b>Both thymoma and T-LGLL could be the cause of secondary PRCA, lymphocyte proliferation may play critical role in the pathogenesis.</p>