Dolor óseo generalizado e hipercalcemia en un lactante mayor como síntoma inicial de linfoma linfoblástico de células B / Generalized bone pain and hypercalcemia in an infant as an initial symptom of lymphoblastic lymphoma of B cells

Resumen Introducción: Los linfomas no Hodgkin son neoplasias heterogéneas derivadas de las células linfohematopoyéticas. Es raro que se presenten antes de los 2 años de edad y la prevalencia es mayor en el sexo masculino. Caso clínico: Paciente de sexo masculino de 1 año y 11 meses que debutó con dolor en miembros inferiores y superiores, claudicación intermitente, deformidad ósea e hipotonía generalizada, por lo que se sospechaba artritis juvenil. Se trató con antiinflamatorios no esteroideos. Al no haber mejoría, ingresó a otro hospital con adenopatías y nodulaciones en la región escrotal y braquial, y hepatomegalia, por lo que se presumió la activación precoz de la pubertad con evidencia de hipercalcemia. Los estudios radiológicos indicaron una posible displasia ósea. Sin embargo, la tomografía por emisión de positrones detectó zonas compatibles con un proceso maligno. Se diagnosticó linfoma de precursores B. La hipercalcemia es una alteración metabólica que, en presencia de cáncer, se considera un síndrome paraneoplásico. Es un dato clínico excepcional que se puede observar en niños con leucemia linfoblástica aguda. Conclusiones: El dolor óseo en la edad pediátrica amerita una exploración física minuciosa para realizar un diagnóstico oportuno del cáncer infantil y mejorar el pronóstico del paciente.

Abstract Background: Non-Hodgkin lymphomas are heterogeneous neoplasms derived from lymphohematopoietic cells, which are rarely found in children < 2 years of age and have a higher prevalence in males. Case report: One-year and eleven-month-old male patient started with pain in the lower and upper limbs, intermittent claudication, bone deformity, and generalized hypotonia, for which juvenile arthritis was suspected. He received non-steroidal anti-inflammatory drug treatment. As no improvement was reported, he was admitted in a different hospital with lymph nodes and nodulations in the scrotal and brachial region and hepatomegaly. Therefore, early activation of puberty with evidence of hypercalcemia was presumed. Radiological studies indicated possible bone dysplasia; however, positron emission tomography detected areas compatible with malignant process. Precursor B lymphoma was diagnosed. Hypercalcemia is a metabolic disorder considered a paraneoplastic syndrome in the presence of cancer, which is an exceptional clinical finding in children with acute lymphoblastic leukemia. Conclusions: Bone pain in the pediatric age deserves a thorough physical examination to favor an early diagnosis of childhood cancer and a better prognosis.

De novo double-hit B-cell precursor leukemia/lymphoma - an unusual presentation as peritoneal lymphomatosis

Peritoneal lymphomatosis (PL) is a rare presentation of extranodal precursor leukemia/lymphoma. The presentation is often non-specific, leading to delayed diagnosis and treatment. In this case, though the preliminary diagnosis was established on ascitic fluid cytology, the disease progressed rapidly, leading to demise before initiating chemotherapy. Immunophenotyping and molecular studies, performed later, established a diagnosis of de novo B-cell precursor leukemia/lymphoma with MYC, BCL2 rearrangements (Double-hit lymphoma). MYC, BCL2 rearrangements are rarely reported in precursor B-lymphoma/leukemia which carry dismal prognosis. In this report, we illustrate autopsy findings of PL in an elderly gentleman who presented with ascites for evaluation.

Clinical, hematological and cytogenetic profile in fibroblast growth factor receptor 1 rearranged hematoloymphoid malignancies

The background of this study is FGFR1 belongs to a family of four, high-affinity receptor tyrosine kinase and is a legitimate oncogene associated with uterine, cervical, prostate, bladder, colorectal and lung cancers. It is rarely concomitant in myeloid and lymphoid neoplasms but has an aggressive clinical course with a high mortality rate when present. Cytogenetic abnormalities involving the FGFR1 gene is most frequently observed in AML, MPN with eosinophilia, T-ALL and T-LBL with ZMYM2 gene being the most common fusion partner. Methods of this study was to authors report a series of 4 cases with FGFR1 rearrangements. Results is three patients presented as T-cell Lymphoblastic lymphoma (T-LBL) and one as mixed phenotype acute leukemia (MPAL). The T-LBL cases harboured the FGFR1/ ZMYM2 fusion and the MPAL case harbored the CNTRL/FGFR1 fusion as identified by conventional cytogenetics and confirmed by molecular studies. Conclusion is authors herewith describe the clinical, biochemical, molecular and cytogenetic features observed in these cases.

Non-Hodgkin's lymphoma of T lymphoblastic type of mediastinum and breast, mimicking thymoma

T cell lymphoblastic lymphoma (T-LBL) is a common variant of Non-Hodgkin’s tumor of mediastinum which presents late due to aggressive spread. Immunohistochemical studies are conclusive in their diagnosis. We present a rare case of mediastinal tumor with left breast mass which mimicked thymoma on computed tomography guided biopsy but was diagnosed T-LBL on immunohistochemistry after surgical excision.

Autologous hematopoietic stem cell transplantation treatment for T cell lymphoblastic lymphoma / 中华血液学杂志

Objective: To investigate the efficacy and predictors of autologous hematopoietic stem cell transplantation (auto-HSCT) in the treatment of T lymphoblastic lymphoma (T-LBL) . Methods: 41 patients with T-LBL who underwent auto-HSCT from April 2006 to July 2017 in the Department of Hematology, the First Affiliated Hospital of Soochow University and the Department of Lymphoma, Peking University Cancer Hospital were analyzed retrospectively. Results: ①Among 41 patients, there were 30 males and 11 females with median age of 24 (11-53) years old. According to the Ann Arbor staging, 33 (80.5%) patients were in stage Ⅲ/Ⅳ. 12 (29.3%) patients have mediastinal involvement, and 20 (48.8%) patients have bone marrow (BM) involvement. Before transplantation, there were 26 (63.4%) patients who achieved first complete remission (CR(1)) , the other 15 (36.6%) patients were in the non-CR(1) group, and there were 29 (70.7%) patients in the low-intermediate risk group (IPI<3 scores) , the other 12 (34.1%) patients were in the middle-high risk group (IPI≥3 scores) . ②The median follow-up was 29 (3-98) months. The 3-year overall survival (OS) and progression-free survival (PFS) for 41 patients were (64.3±8.2) % and (66.0±7.8) %, respectively. 3-year cumulative recurrence rate (CIR) was (30.7±7.4) %, and 3-year non-recurring mortality (NRM) was (4.8±4.6) %. ③The 3-year OS of the CR(1) group and the non-CR(1) group were (83.4±7.6) % and (38.9±12.9) % (P=0.010) , and the 3-year PFS of two groups were (83.8±7.4) % and (40.0±12.6) % (P=0.006) , respectively. The 3-year CIR of these two groups were (16.2±7.4) % and (53.3±12.9) % (P=0.015) , and the 3-year NRM were 0 and (14.3±13.2) % (P=0.157) , respectively. ④The 3-year OS of the IPI low-intermediate risk group and the high-intermediate risk group were (76.9±8.4) % and (35.7±15.2) % (P=0.014) and the 3-year PFS were (77.4±8.2) % and (40.0±14.6) (P=0.011) , respectively. The 3-year CIR of these two groups were (18.1±7.3) % and (60.0±14.6) % (P=0.006) , and the 3-year NRM were (5.6±5.4) % and 0 (P=0.683) , respectively. The OS and PFS of patients with low-intermediate risk group were significantly higher than the other group. Conclusion: Auto-HSCT could improve the survival of T-LBL. Pre-transplant status and IPI score are important predictors for survival T-LBL patients with auto-HSCT.

Extramedullary CD20-positive B-lymphoblastic lymphoma in a 5-year-old child: A diagnostic challenge

@#Lymphoblastic leukaemia/lymphoma may present as an isolated extramedullary mass, which includes the musculoskeletal region involvement with normal or near-normal blood counts. The tumour may be in the form of B or T-lymphoblastic leukaemia/lymphoma. The clinical features and histological morphology of extramedullary B-lymphoblastic lymphoma (B-LBL) may mimic mature B-cell neoplasms, thus posing a diagnostic challenge. Arriving at the right diagnosis is crucial because these two diseases differ in their prognosis and management. A high index of suspicion is therefore important so as not to miss the correct diagnosis. The diagnosis may be overlooked because the clinical presentation may not be typical of B-LBL or the blood counts do not show any abnormalities. In this report, we highlight one such case where the diagnosis of B-LBL was missed because of its atypical presentation.

Study progress of T-lymphoblastic lymphoma in children / 中华实用儿科临床杂志

Lymphoblastic lymphoma (LBL) is a rare aggressive neoplasm of T-/B-precursors resembling acute lymphoblastic leukemia,which develops very fast with high morbidity.T-lymphoblastic lymphoma (T-LBL),accounting for 85％-90％ of LBL,develops more frequently in children and young adults and is typically characterized by a grossly enlarged mediastinum,whose diagnostic hallmark is the expression of a T-precursor cell immunophenotype.Recently many new diagnostic technologies,such as polymerase chain reaction,flow cytometry,fluorescence in situ hybridization and so on,have been used for the dignosis of LBL,which lay the foundation of the precision therapy for LBL.The adoption of pediatric-derived,intensive lymphoblastic leukemia-like protocols leads to significantly improved results,with event free survival about 75％-90％ in children.New clinical trials will introduce and confirm the value of new drugs and targeting agents,which may have more good effects.New minimal residual disease assessment methods(eg,next generation sequencing,NGS) make it possible to detect MRD in different subtype of childhood lymphomas.Now,the new advances in diagnosis and treatment of T-LBL were reviewed.

Clinicopathological characterization of non-Hodgkin lymphoma of the prostate / 中华男科学杂志

Objective@#To study the clinicopathological characteristics of non-Hodgkin lymphoma (NHL) of the prostate.@*METHODS@#We collected the clinical data on 6 cases of NHL of the prostate pathologically confirmed between 2001 and 2017. The patients were aged 49－76 (median 62) years old, with the main clinical manifestations of painless swelling of the prostate and lower urinary tract obstruction. We analyzed the clinical features and the results of histological detection, immunohistochemical staining and B-cell gene rearrangement assay, and explored the clinicopathological characteristics and differential diagnosis of the disease based on the relevant literature.@*RESULTS@#Histological detection revealed diffuse large B-cell lymphoma (DLBCL) in 4 cases (66.7%), B-lymphoblastic lymphoma (B-LBL) in 1 (16.7%), and Burkitt lymphoma (BL) in another (16.7%). DLBCL was histologically characterized by diffuse oval or round medium-to-large-sized lyphoid cells with an infiltrative growth pattern, B-LBL by monotonous small-to-medium-sized lymphoid cells with prominet mitosis and apoptosis, and BL by diffuse and monotonous medium-sized neoplastic cells with round or oval nuclei, an infiltrative growth pattern, scanty cytoplasm and visible mitosis. One of the DLBCL patients received 5 doses of R-CHOP chemotherapy and has been followed up to the present time, while the other 3 were lost to follow-up; the B-LBL patient died at 1 month after diagnosis; and the BL patient gave up treatment.@*CONCLUSIONS@#Non-Hodgkin's lymphoma of the prostate mostly presents as diffuse large B-cell lymphoma, and its diagnosis depends on immunohistochemistry and related molecular detection as well as its clinical and histopathological manifestations.

Recurrent or refractory childhood B cell lymphoblastic lymphoma:A case report and literature review / 吉林大学学报(医学版)

Objective:To explore the clinical characteristics,the diagnostic framework,and the treatment methods of B cell lymphoblastic lymphoma(B-LBL),and to clarify the progress of diagnosis and treatment of B-LBL to improve the clinician's understanding of the disease and provide the guidance for prognostic evaluation and therapeutic options.Methods:The clinical data including symptoms,physical signs,ancillary testings,diagnosis, treatment and disease prognosis of a child suffered from B-LBL were retrospectively analyzed;in the meantime,the relative literatures were reviewed.Results:The patient was definitly diagnosed as B-LBL according to the clinical characteristics and received combination therapy with vincristine,daunorubicin,L-asparaginase,and prednisone as the first course,along with the intrathecal injection of methotrexate and dexamethasone to prevent central nervous system leukemia(CNS-L).The patient achieved complete remission(CR)25 d after the first circle chemotherapy but was diagnosed as degree 4 myelosuppression.Therefore,the second cycle combination therapy was adjusted with cyclophosphamide,cytarabine and 6-MP,and the intrathecal injection to prevent CNS concomitantly.DegreeⅣ myelosuppression appeared repeatedly after 2 cycles and the combination chemotherapy was reajdusted. So mercaptopurine and high dose of methotrexate were given as the 4th cycle,and CNS was prevented continously. The patient kept CR until the second cycle finished but get recurrence after the third chemotherapy(prolymphocytes 10%).Then remission and recurrence were found in the disease counrse during which mary chemotherapy methods were attempted until the patient got stable CR after treatment for 31 months.Then the patient was treated with oral mercaptopurine(50 g·d-1)and methotrexate(25 mg per week)and kept disease-free survival for more than 3 years.Conclusion:B-LBL is a rapidly developed disease with the bone marrow involvement occurring in the short term and easy to relapse during treatment.However,it is extremely easy to transform to recurrent and refractory B-LBL after the first remission.It is of great importance to estimate the risk stratification and to evaluate the prognosis of LBL patients in order to treat as soon as possible for the improvement of one's life quality and the prolongation of survival.

Recurrent or refractory childhood B cell lymphoblastc lymphoma: A case report and literature review / 吉林大学学报(医学版)

Objective: To explore the clinical characteristics, the diagnostic framework, and the treatment methods of B cell lymphoblastic lymphoma (B-LBL), and to clarify the progress of diagnosis and treatment of B-LBL to improve the clinician's understanding of the disease and provide the guidance for prognostic evaluation and therapeutic options. Methods: The clinical data including symptoms, physical signs, ancillary testings, diagnosis, treatment and disease prognosis of a child suffered from B-LBL were retrospectively analyzed; in the meantime, the relative literatures were reviewed. Results: The patient was definitly diagnosed as B-LBL according to the clinical characteristics and received combination therapy with vincristine, daunorubicin, L-asparaginase, and prednisone as the first course, along with the intrathecal injection of methotrexate and dexamethasone to prevent central nervous system leukemia (CNS-L). The patient achieved complete remission (CR) 25 d after the first circle chemotherapy but was diagnosed as degree 4 myelosuppression. Therefore, the second cycle combination therapy was adjusted with cyclophosphamide, cytarabine and 6-MP, and the intrathecal injection to prevent CNS concomitantly. Degree IV myelosuppression appeared repeatedly after 2 cycles and the combination chemotherapy was reajdusted. So mercaptopurine and high dose of methotrexate were given as the 4th cycle, and CNS was prevented continously. The patient kept CR until the second cycle finished but get recurrence after the third chemotherapy (prolymphocytes 10%). Then remission and recurrence were found in the disease counrse during which Mary chemotherapy methods were attempted until the patient got stable CR after treatment for 31 months. Then the patient was treated with oral mercaptopurine (50 g · d-1) and methotrexate 25 mg per week) and kept disease-free survival for more than 3 years. Conclusion: B-LBL is a rapidly developed disease with the bone marrow involvement occurring in the short term and easy to relapse during treatment. However, it is extremely easy to transform to recurrent and refractory B-LBL after the first remission. It is of great importance to estimate the risk stratification and to evaluate the prognosis of LBL patients in order to treat as soon as possible for the improvement of one's life quality and the prolongation of survival.

Detection of immunoglobulin and TCR gene rearrangements by PCR using BIOMED-2 multi-plex protocols in T lymphoblastie lymphoma and acute lymphoblastic leukemia / 临床与实验病理学杂志

Purpose To investigate the sensitivity of BIOMED-2 primer system in T lymphoblastic lymphoma ( T-LBL) and acute lym-phoblastic leukemia ( ALL) patients immunoglobulin ( Ig) and T-cell receptor ( TCR) gene rearrangement, and to analyze the co-rear-rangement pattern. Methods Amplification of rearranged Ig and TCR gene was performed in standard PCR in 35 T-LBL/ALL pa-tients. PCR products were analyzed by heteroduplex and polyacrylamide gel electrophoresis. Results 16 cases (45. 7%) of 35 sam-ples were detected to have TCR gene rearrangements, including 6 cases (37. 5%) of TCRβgene monoclonal rearrangements, 4 cases (25. 0%) of TCRγ gene monoclonal rearrangements, 3 cases (18. 8%) of TCRβ and TCRγ gene double rearrangements, 2 cases (12. 5%) of TCRδ gene monoclonal rearrangements and 1 case (6. 3%) of TCRγand TCRδgene double rearrangements were detec-ted. 4 cases (11. 4%) of 35 samples detected to have clonal immunoglobulin and TCR gene rearrangements. 11 cases (39. 3%) of 28 T-LBL patients were detected to have TCR gene rearrangements, 6 cases (85. 7%) of 7 T-ALL have TCR gene rearrangements. Con-clusions BIOMED-2 multiplex PCR analysis strategy is a useful technique in the T-LBL patients.

Case series of precursor B-cell lymphoblastic lymphoma

Precursor B-cell lymphoblastic lymphoma (B-LBL) is an uncommon subtype of Non-Hodgkin lymphoma (NHL), accounting for only 0.3% of NHL in adults and less than 10% of all LBL cases. Unlike T-cell LBL, it usually presents with extranodal involvement while sparing the bone marrow (BM). Among the 27 patients with LBL treated in the Asan Medical Center between January 2007 and March 2012, 3 had B-LBL. All had a good performance status and low International Prognostic Index. However, unlike most previously reported cases, the patients had lymphoma in their bone marrow and extranodal sites such as bone and lung. After intensive combination chemotherapy, one patient achieved a complete response and the other 2 patients, a partial response. Our experience suggests that multiple extranodal sites may be involved in B-LBL and BM involvement may not be as infrequent as previously thought. Furthermore, intensive chemotherapy seems to be effective.

Cutaneous Involvement of T-cell Lymphoblastic Lymphoma / 대한피부과학회지

Lymphoblastic lymphoma is classified as non-Hodgkin's lymphoma with high tendency of rapid progression to acute leukemia. Cutaneous involvement of this condition is rare. The clinical feature of skin lesions usually present single or multiple papular or nodular lesions preferentially located on the head and neck. Herein, we report a case of an 18-year-old man presenting skin involvement of T cell lymphoblastic lymphoma. The lesions manifested as asymptomatic, multiple, round, slightly pinkish nodules on the scalp and trunk. Histological examination showed dense, diffuse infiltration of medium-sized lymphoid cells throughout the entire dermis. The neoplastic cells had scant cytoplasm and nuclei characterized as round or slightly irregular with expression of CD3, CD5, CD45, TdT and Ki-67.

Solitary Lymphoblastic Lymphoma of the Thoracic Spine

Non-Hodgkin's lymphoma rarely originates from bone, and even more infrequently from a vertebral body. Lymphoblastic lymphoma is a rare type of non-Hodgkin's lymphoma, and results from an abnormality in adaptive immune cells. A 27-year-old man presented with a two-month history of night sweats, weight loss, and severe back pain. Radiological studies demonstrated an osteolytic lesion compressing the subarachnoid space at the T11 level. Posterolateral fusion with decompression was performed and a pathologic examination confirmed lymphoblastic lymphoma of the B-cell precursor type. To our knowledge, this is the first report of solitary lymphoblastic lymphoma from B-cell precursors in of the thoracic spine. Herein, we discuss the presenting symptoms and the management of this rare case of lymphoblastic lymphoma.

Simultaneous isolated bilateral facial palsy : A rare vincristine-associated toxicity.

We describe a case of a 15-year-old boy with vincristine-induced simultaneous isolated bilateral facial palsy. The boy presented with superior vena caval syndrome (SVC syndrome), right-sided pleural effusion and anterior mediastinal lymphadenopathy. Histopathological examination of left axillary lymph node was suggestive of lymphoblastic lymphoma. We started chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisolone. SVC syndrome disappeared completely after the 1st cycle, and he achieved remission after the 3rd cycle of chemotherapy. He noticed that he could not close his eyes. Neurological examination revealed bilateral lower motor neuron facial palsy. Findings from examination of other cranial nerves and peripheral nerves were normal. Results of MRI of brain and cerebrospinal fluid examination were normal. He received 6 mg vincristine before developing toxicity.

T- Lymphoblastic lymphoma in adults / Linfoma linfoblástico T dos adultos

Adult T-lymphoblastic lymphoma is rare and has a poor prognosis. In the 80s, following the introduction of sequential, intensified chemotherapy, complete remissions in the order of 75 percent-95 percent of treated patients, were achieved. However, several patients, namely those with advanced disease, continued to relapse either in remission or during maintenance therapy. Moreover, all these early studies were not able to detect any valuable prognostic index to predict the outcome. In an attempt to reduce the relapse rate, upfront autologous stem cell transplantation in patients in complete remission was introduced. The results obtained with this approach were quite homogeneous, indicating a probability of disease-free survival of about 65 percent-75 percent and an overall survival rate of 60 percent. Successive therapies designed since 2000 were able to obtain complete remissions of above 90 percent, with a relapse rate in the order of 30 percent and an overall survival comparable to that obtained with the transplant procedure. Yet, these studies were also unable to detect valuable prognostic factors predictive of the outcome. Moreover, no study on the biologic profile of the disease has been developed. To improve the prognosis of T-lymphoblastic lymphoma it seems necessary to create national registries to collect both clinical and biological data of all lymphoblastic lymphoma patients. In this way it will be possible to reach critical numbers of data with which valid statistical analysis may be performed that is able to detect factors influencing the outcome. Moreover, subsets of patients needing intensified procedures such as stem cell transplant may be detected at diagnosis.

O linfoma linfoblástico de célula T é raro e com prognóstico ruim. Após introdução de terapêutica quimioterápica seqüencial e intensificada, remissões completas passaram a ser obtidas em 75 por cento-95 por cento dos pacientes. Entretanto, muitos pacientes, particularmente aqueles com a chamada doença avançada, continuaram a recair tanto durante a terapia de indução como na manutenção. Além disso, todos estes estudos iniciais não foram capazes de detectar qualquer índice prognóstico capaz de prever a evolução dos pacientes. No sentido de reduzir as taxas de recidiva, o transplante autólogo de célula progenitora hematopoética em pacientes em remissão completa foi introduzido. Os resultados obtidos com esta abordagem foram bastante homogêneos, indicando uma probabilidade de sobrevida livre de doença de 65 por cento-75 por cento e uma sobrevida global de 60 por cento. Sucessivos tratamentos desenhados já nos anos 2000, foram capazes de obter remissões completas acima de 90 por cento, com taxas de recidivas da ordem de 30 por cento e uma sobrevida global comparável à obtida com o transplante. Ainda, estes estudos também não foram capazes de detectar fatores prognósticos relacionados à evolução válidos. Mais ainda, qualquer estudo com perfil biológico foi desenvolvido. Para melhorar o prognóstico do LLB-T parece ser necessário esforço multicêntrico, de caráter nacional ou internacional, para coletar dados clínicos e biológicos. Nesta linha, é possível alcançar número crítico de dados com valor estatístico que poderiam ser capazes de detectar fatores com influência prognóstica. Finalmente, grupos de pacientes necessitam ser identificados para selecionar aqueles que poderiam se beneficiar do transplante de célula progenitora hematopoética detectados ao diagnóstico.

Dificuldade diagnóstica em dois casos de neoplasia de células linfóides precursoras cutâneas primárias / Diagnostic pitfalls in two cases of primary cutaneous neoplasia of precursor lymphocytes

O linfoma linfoblástico (LLB) é uma neoplasia maligna de linfócitos precursores (B, T ou células NK). O comprometimento primário da pele é raro. Relatamos as características clínicas, anatomopatológicas e imunofenotípicas em dois pacientes com apresentação cutânea primária que, histologicamente, apresentavam infiltrado de células imaturas. A análise imunofenotípica foi realizada com amplo painel de anticorpos. A pesquisa de rearranjo no gene do receptor de células T (TCR-gama) pelo método de reação em cadeia da polimerase (PCR) resultou positiva em um caso, que era CD56 positivo, classificado como linfoma de células NK blásticas-símile. Este caso representa uma entidade distinta derivada de células precursoras num estágio precoce de uma via comum de diferenciação para células T e NK. O outro caso foi classificado como LLB-T com expressão aberrante de CD79a, o que poderia ser erroneamente interpretado. O diagnóstico correto depende da utilização de um amplo painel de anticorpos para caracterização imunofenotípica e avaliação molecular.

The lymphoblastic lymphoma (LBL) is a malignant neoplasm of precursor lymphocytes (B, T or NK-cells). The primary involvement of the skin is rare. We examined the clinical, anatomopathological and immunophenotypic features of two patients with primary cutaneous involvement. Histologically they showed an infiltrate of immature cells. The immunophenotypic analysis was performed with a comprehensive panel of antibodies. T-cell receptor rearrangement (TCR-gamma) was analyzed with polymerase chain reaction (PCR) and it was positive in one case, which was CD56 positive, classified as blastic NK-cell-like lymphoma. This case represents a distinct entity derived from precursor cells at an early stage of a common developmental pathway for T and NK cells. The other case was classified as T-cell lymphoblastic lymphoma with aberrant expression of CD79a, what could be a diagnostic pitfall. The accurate diagnosis depends on the use of a comprehensive panel of antibodies for immunophenotypic characterization and molecular analysis.

La citometría de flujo en el estudio de tumores mediastinales ricos en elementos linfoides / Flow cytometry for the study of mediastinal tumors with abundant lymphoid elements

El mediastino anterior es un sitio frecuente de localización de tumores ricos en elementos linfoides. La identificación correcta de cada entidad es de importancia en el tratamiento de los pacientes. En ocasiones puede plantearse el diagnóstico diferencial entre timoma y linfoma linfoblástico con fenotipo de precursor T (LLB-T). La Citometría de Flujo (CF) es una técnica complementaria útil para estos tumores de la cual se obtiene información cualitativa y cuantitativa. Revisamos 38 tumores mediastinales que tenían estudio de CF. Además comparamos los resultados de CF de timomas y tejido tímico normal con 42 casos de LLB-T de otras localizaciones anatómicas. De los 38 tumores mediastinales 6 eran lesiones benignas, 9 linfomas difusos de células grandes con fenotipo B (LDCG-B), 10 linfomas de Hodgkin (LH), 11 timomas y 2 LLB-T. En 24 casos la CF aportó información positiva, definiendo el inmunofenotipo de las células linfoides neoplásicas, o los linfocitos característicos que acompañan a los timomas. La CF en los 10 casos de LH y en 4 lesiones benignas permitió descartar otros tipos de linfoma (LDCG-B, LLB-T, etc.). Las marcaciones para CD3, CD4 y CD8 fueron las más útiles para el diagnóstico diferencial entre timomas y LLB-T. En conclusión, la CF es una técnica complementaria de utilidad que aporta información en lesiones mediastinales de manera rápida, requiriendo cantidades pequeñas de material, tanto para el diagnóstico inicial como para el monitoreo de estas enfermedades.

The anterior mediastinum is a common site of tumors with abundant lymphoid elements. Flow cytometry is a useful complementary technique to analyze this type of tumors, which provides qualitative and quantitative information. A differential diagnosis can be sometimes made between thymoma and precursor T-lymphoblastic lymphoma (T-LBL). Correct identification is of utmost importance for patient treatment. A total of 38 mediastinal tumors were analyzed, and samples were separated for flow cytometry. Flow cytometry data from thymomas and normal thymic tissue were compared with 42 cases of T-LBL from other anatomical locations. Among 38 mediastinal tumors, we found 6 benign lesions, 9 diffuse large B-cell lymphomas (DLBCL), 10 Hodgkin lymphomas (HL), 11 thymomas and 2 T-LL. Flow cytometry provided positive information in 24 cases, and defined lymphoid neoplastic cells immunophenotype or the typical lymphocytes accompanying thymomas. Flow cytometry helped differentiate 10 cases of HL and 4 benign lesions from other lymphomas (DLBCL, T-LBL, etc.). CD3, CD4 and CD8 expressions were most useful for the differential diagnosis of thymomas and T-LL. To conclude, flow cytometry is a valid complementary technique, which promptly provides information on mediastinal lesions, requiring small quantities of tissue for both early diagnosis and follow up of these diseases.

A Case of Cutaneous Involvement of T-cell Lymphoblastic Lymphoma / 대한피부과학회지

Lymphoblastic lymphoma is a malignant neoplasm of precursor lymphocytes with a B- or T cell phenotype. T cell lymphoblastic lymphoma is a clinically aggressive disease with frequent involvement of extranodal sites, but the involvement of the skin is rare. The clinical appearance of skin lesions usually includes single or multiple papular or nodular lesions preferentially located on the head and neck. Herein, we report the case of a 31-year-old man presenting with skin involvement of T cell lymphoblastic lymphoma. The lesions manifested as asymptomatic, multiple, round, reddish brown macules, and patches on both extremities. Histology examination showed dense, diffuse infiltration of medium sized lymphoid cells into the entire dermis. The tumor cells had irregular nuclei, finely dispersed chromatin, inconspicuous nucleoli, scant cytoplasm, and expressed TdT, CD3 and CD5.

A Case of Precursor B-cell Lymphoblastic Lymphoma Misdiagnosed as Rosacea / 대한피부과학회지

Precursor B-cell lymphoblastic lymphoma is a rare type of non-Hodgkin's lymphoma seen exclusively in children and young adults. The neoplasm is rare in old age. We report a case of a 58-year-old male, who presented with variable-sized, erythematous to brownish papules and plaques on the scalp and face. There were no other symptoms. Pathological examination showed non-specific, sparse superficial and deep perivascular lymphocyte infiltration. We diagnosed the condition as rosacea and prescribed 100 mg of minocycline per day for 10 days. When he was seen 10 days later, his skin lesions were aggravated and re-biopsy was completed. Histopathology revealed diffuse infiltrates of small to medium-sized lymphoid cells with blastic nuclear chromatin and a high mitotic rate. Immunohistochemical study showed that infiltrated lymphoid cells were precursor B-cell type. Physical examination and staging work-up revealed extensive involvement of lymphoma in bilateral kidney, heart, pancreas, axial and proximal appendicular bones, scalp and cervical lymph node. The patient is being treated with combination chemotherapy.