Introduction on the update of the 5th edition WHO classifications of B-cell neoplasms / 中华病理学杂志

The 5th edition WHO classification of B-cell tumors is a systematic update to the fourth revised version of the classification. The changes include updated names of entities, sharpened diagnostic criteria, and upgrades from provisional to definite entities. This review focuses on the changes in the content of each chapter of B-cell tumors, facilitating domestic colleagues engaged in the diagnosis and treatment of lymphohematopoietic tumors to understand the latest progress and guide daily work.

The Expression Level and Diagnostic Value of Serum Free Light Chain in B-Cell Non-Hodgkin Lymphoma / 中国实验血液学杂志

OBJECTIVE@#To investigate the expression level and the diagnostic value of serum free light chain in B-cell non-Hodgkin's lymphoma (B-NHL).@*METHODS@#We retrospectively analyzed the results of serum free light chain (sFLC) of 394 newly treated B-NHL patients in our hospital from January 2014 to December 2021 and compared the secretion levels of sFLC among different subtypes of B-NHL. The value of sFLC secretion levels in the diagnosis of WM was evaluated using ROC.@*RESULTS@#Increased proportion of sFLC, abnormal ratio of sFLC (κ / λ) and the secretion levels of sFLC (κ+λ) were different in different B-NHL subtypes, Waldenstrom's macroglobulinemia (WM) had the highest proportion of elevated sFLC(82.68%) and abnormal sFLC(κ/ λ)(87.0%), the proportion of FL(18.0%) and DLBCL patients(12.8%) with elevated sFLC was lower (P<0.05). The expression levels of sFLC can helpful in the diagnosis of WM (AUC=0.874，P<0.001， 95% CI: 0.779-0.970). At the same time, higher sFLC levels and sFLC cloning patterns predicted the possibility of bone marrow infiltration of lymphoma.@*CONCLUSION@#The serum free light chains is common in patients with B-NHL. The elevated level and type of free light chain are associated with the type of lymphoma, and the patients with bone marrow infiltration have higher sFLC（κ+ λ） expression level.

Intravascular large B-cell lymphoma: The Great Imitator

Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of diffuse large B-cell lymphoma with an estimated incidence of less than one per million. Unlike other hematopoietic malignancies, lymphadenopathy and hepatosplenomegaly are uncommon, and patients typically present with nonspecific symptoms. IVLBCL presents a diagnostic challenge and patients are usually diagnosed late in the disease course, if at all, and the prognosis is poor. The differential diagnosis is broad, and physicians often pursue multiple diagnostic possibilities during patient workup. We present a case of IVLBCL discovered at autopsy in an 80-year-old male who presented with history and symptoms pointing to the tick-borne illness ehrlichiosis.

Bilateral Severe Vitritis, an intraocular recurrence of B cell lymphoma: a case report / Vitritis severa bilateral, una recurrencia intraocular de linfoma de células B: reporte de caso

Purpose: To report a case of bilateral vitritis originated from Primary Central Nervous System Lymphoma. Diagnosis was made from a careful history taking and confirmed with vitrectomy. Case report: 65-year-old Vietnamese male had one month of progressive blurred vision in both eyes without other eye complaint. History revealed that about one year previously, he was diagnosed with primary diffuse large B cell lymphoma. He was treated with chemotherapy of Methotrexate 3.5 g/m2and cytarabine 2 mg/m2, and a whole brain radiation therapy. There was a complete remission after the therapy. Fundus of both eyes was partly obscured by cells mainly in the posterior vitreous. Sequential bilateral vitrectomies were done on 5/1/17 and 7/17/17 without complication. The vitreous sample from the first eye having vitrectomy was sent for study which demonstrated large B cell lymphoma. Post-operative vision improved to 20/25 both eye without the complaint of hazy vision. Conclusion: Good history taking assisted in the diagnosis of intraocular lymphoma in this case with bilateral vitritis. However, bilateral vitrectomy restored the vision and further confirmed the diagnosis of lymphoma for future follow up care

Objetivo: Reportar un caso de vitritis bilateral originada por un Linfoma del Sistema Nervioso Central Primario. El diagnóstico se realizó a partir de una cuidadosa historia clínica y confirmada con vitrectomía. Reporte de caso: Varón vietnamita de 65 años con un mes de visión borrosa progresiva en ambos ojos sin otra afección ocular. La historia reveló que hace aproximadamente un año, se le diagnosticó un linfoma difuso primario de células B grandes. Fue tratado con quimioterapia con Methotrexate 3.5 g/m2 y citarabina 2 mg/m2 y una radioterapia cerebral completa. Hubo una remisión completa después de la terapia. El fondo de ambos ojos estaba parcialmente oscurecido por las células, principalmente en el vítreo posterior. Las vitrectomías bilaterales secuenciales se realizaron el 5/1/17 y el 17/7/17 sin complicaciones. La muestra vítrea del primer ojo sometida a vitrectomía fue enviada a un estudio que demostró linfoma de células B grandes. La visión postoperatoria mejoró a 20/25 ambos ojos sin la queja de la visión borrosa. Conclusión: una buena historia clínica fue útil en el diagnóstico de linfoma intraocular en este caso con vitritis bilateral. Sin embargo, la vitrectomía bilateral restauró la visión y confirmó aún más el diagnóstico de linfoma para el cuidado de seguimiento futuro.

Linfoma cutáneo primario de células B centrofolicular: un desafío diagnóstico / Primary cutaneous follicular B-cell lymphoma: a diagnostic challenge

El linfoma cutáneo primario de células B (LCPCB) centrofolicular corresponde a una proliferación neoplásica infrecuente e indolente de células del centro germinal confinadas a la piel. Se reporta y revisa un caso de LCPCB centrofolicular. Paciente femenino de 76 años, con antecedentes de hipertensión arterial y resistencia a la insulina. Consultó por aumento de volumen frontal de dos años de evolución. Al examen físico destacaba un nódulo único en región frontal derecha. La ecografía de partes blandas fue compatible con quiste epidérmico. La histopatología demostró en dermis profunda, tejido adiposo subcutáneo y tejido muscular estriado una proliferación linfoide sólida dispuesta en patrón nodular y difuso. A la inmunohistoquímica (IHQ), los linfocitos fueron CD20 y BCL-6 positivo, con un Ki-67 de 60% y BCL-2, CD3, CD5 y CD10 negativo. El estudio de diseminación tumoral resultó negativo. El LCPCB centrofolicular concentra el 60% de todos los LCPCB. Se presenta en promedio a los 51 años. Se ha descrito asociación con Borrelia burgdorferi, VIH, virus hepatitis C y virus Epstein-Barr. Clínicamente corresponde a un nódulo eritematoso y asintomático, localizado preferentemente en cabeza, cuello y tronco. La IHQ es fundamental para diferenciarlo de otros tipos de LCPCB. Habitualmente, el tratamiento es con radioterapia o cirugía escisional. La supervivencia es de un 95% a 5 años. Se presenta este caso dado que el LCPCB centrofolicular corresponde a un tumor cutáneo infrecuente, con múltiples diagnósticos diferenciales, que requiere de un alto índice de sospecha para lograr un diagnóstico y tratamiento oportuno.

The primary cutaneous follicle center lymphoma (PCFCL) corresponds to an infrequent and indolent neoplastic proliferation of germinal center cells confined to the skin. A case of PCFCL is reported and revised. Results: A female patient, 76 years old, with arterial hypertension and insulin resistance. Sough attention for an increase in size of the frontal region over the course of two years. Upon physical examination, a single nodule was noted in the right frontal region. A soft tissue ultrasound identified results indicative of an epidermal cyst. The histopathology revealed a proliferation of solid lymphoid arrayed in a diffuse and nodular pattern in the deep dermis, subcutaneous adipose tissue, and striated muscular tissue. Immunohistochemistry (IHQ) revealed CD20 and BCL-6 positive lymphocytes, with 60% of Ki-67 and BCL-2, CD3, CD5, and CD10 negative. A study of tumor dissemination resulted negative. The PCFCL concentrates 60% of all primary cutaneous B cell lymphomas (PCBCL). It presents at an average of 51 years of age. It has been described association with Borrelia burgdorferi, HIV, hepatitis C virus and Epstein-Barr virus. Clinically it corresponds to an erythematous and asymptomatic nodule, found frequently on the head, neck and trunk. The IHQ is essential to differentiate it from other types of PCBCL. It is usually treated with radiotherapy or excisional surgery. Survival is 95% over 5 years. This case is presented because the PCFCL corresponds to an infrequent cutaneous tumor, with multiple differential diagnoses, requiring a high index of suspicion to achieve an opportune diagnostic and treatment.

Cutaneous primary B-cell lymphomas: from diagnosis to treatment

AbstractPrimary cutaneous B-cell lymphomas are a heterogeneous group of mature B-cells neoplasms with tropism for the skin, whose biology and clinical course differ significantly from the equivalent nodal lymphomas. The most indolent forms comprise the primary cutaneous marginal zone and follicle center B-cell lymphomas that despite the excellent prognosis have cutaneous recurrences very commonly. The most aggressive forms include the primary cutaneous large B-cell lymphomas, consisting in two major groups: the leg type, with poor prognosis, and others, the latter representing a heterogeneous group of lymphomas from which specific entities are supposed to be individualized over time, such as intravascular large B-cell lymphomas. Treatment may include surgical excision, radiotherapy, antibiotics, corticosteroids, interferon, monoclonal antibodies and chemotherapy, depending on the type of lymphoma and on the type and location of the skin lesions. In subtypes with good prognosis is contraindicated overtreatment and in those associated with a worse prognosis the recommended therapy relies on CHOP-like regimens associated with rituximab, assisted or not with local radiotherapy. We review the primary cutaneous B-cell lymphomas, remembering the diagnostic criteria, differential diagnosis, classification, and prognostic factors and presenting the available therapies.

Pediatric B-cell Lymphoma, Unclassifiable, With Intermediate Features Between Those of Diffuse Large B-cell Lymphoma and Burkitt Lymphoma: A Report of Two Cases

No abstract available.

Primary Intestinal Lymphoma: A Clinicopathological Study.

Introduction: Primary intestinal lymphoma (PIL) is defined as an extranodal lymphoma arising in the intestine. This study includes 11 PILs. Objectives: The objective of this study was to evaluate the clinicopathological characteristics of primary non-Hodgkin's lymphoma (NHL) in the small and large intestine. All cases were reclassified according to the World Health Organization classification of lymphoma in 2001. Immunohistochemistry (IHC) was used to confirm the histopathological diagnosis. Materials and Methods: Eleven cases of primary non-Hodgkin in the small and large intestine were studied retrospectively in a 5 year period. There were seven cases of resected intestinal specimens and four biopsy specimens. Five cases from ileum and two cases each from caecum and duodenum and one case each from jejunum and duodenum. Hematoxylin and eosin stained sections were studied with light microscopy and IHC for CD5, 20, 21 and cyclin D1 were done. Results: It is a retrospective study of 11 cases of intestinal lymphomas. Abdominal pain and abdominal lump were two main common presenting symptoms. NHLs were more common in the small intestine. PILs are common in adults (75% above 46 years) with male preponderance. All were NHLs and B-cell type. Five cases were diffuse large B-cell lymphoma and four cases each were mucosa-associated lymphatic tissue lymphoma and follicular lymphoma. One case was lymphomatoid polyposis or mantle cell lymphoma. Conclusion: PILs is rare and differs significantly from their gastric counterpart, not only in pathology but also with regard to clinical features, management and prognosis. Due to the lack of characteristic symptoms and a low incidence rate, PIL is misdiagnosed until serious complications occur, such as perforation and bleeding and hence needs to be accurately diagnosed.

Linfoma primario B tipo malt en glándula tiroides / B type primary malt lymphoma in thyroid gland

Los linfomas primarios de tiroides representan menos del 5% de las neoplasias primarias con elevada asociación con la tiroiditis de Hashimoto y tiroiditis linfocítica. Son tipos histológicos más frecuentes el linfoma B difuso de células grandes y el linfoma B de la zona marginal tipo MALT. Se presenta el caso de una paciente de 46 años que acudió a consultorio por aumento de volumen en región anterior de cuello, asfixia y ronquera. Con el diagnóstico de linfoma tiroideo, la paciente fue sometida a tiroidectomía total. El informe anatomopatológico confirmó el diagnostico de linfoma B de la zona marginal tipo MALT asociado a tiroiditis de Hashimoto, con positividad por inmunohistoquímica para marcadores linfoides B CD20 y CD21.

Primary thyroid lymphomas represent less than 5% of primary tumors with high association with Hashimoto’s thyroiditis and lymphocytic thyroiditis. Most common histological types are diffuse large B-cell lymphoma and lymphoma of MALT type B marginal zone. The case of a 46 year old who came to office by increased volume in anterior neck, choking and hoarseness. With the diagnosis. Thyroid lymphoma . The patient underwent total thyroidectomy. The pathology report confirmed the diagnosis of B lymphoma marginal zone MALT associated with Hashimoto’s thyroiditis, with positivity by immunohistochemistry for B lymphoid markers CD20 and CD21.

Linfomatosis intravascular cerebral como causa de demencia rápidamente progresiva: reporte de un caso / Cerebral intravascular lymphomatosis as cause of subacute dementia: report of a case

Cerebral Intravascular Lymphomatosis is a type of non Hodgkin Lymphoma, generally composed with B cells, its ocurrence is infrequent, clinically progressive and has a fatal course. Vascular damage is related with tumoral infiltration and small caliber occlusion, with results in multi infarcts. Neurological symptoms and signs are frequently the first clinical manifestation, which include sub acute encephalopathy, cognitive impairment, delirium, aphasia, hemipharesis, visual disturbances, paraplegia, paresthesia and cranial nerves involvement. MRI shows images of vasculitis from CNS. Cerebral Intravascular Lymphomatosis must be considered in differential diagnosis of rapid and subacute dementias, and clinical cases with small vessel recurrent multi infarct of unusual etiology. We present the clinical case of a patient with rapid progressive dementia and systemic disease manifestation. The CNS involvement was characterized as a subacute encephalopathy, with confusion and agitation, seizures, motor disturbances, bilateral plantar extensor reflexes and cerebellar signs. The clinical symptomatic course was progressive, with weight loss and fluctuant fever. The patient had a fatal course after he was treated with Methylprednisolone. Postmortem pathologic examination revealed a diffuse non Hodgkin lymphoma of B cells, intravascular variant, with brain compromise, cerebellum, suprarenal glands, pancreas, myocardium, thyroid gland, lung kidney and the lever...

La linfomatosis intravascular cerebral es un tipo de Linfoma no Hodgkin generalmente de células B, de presentación infrecuente, curso clínico progresivo y fatal. El daño vascular es producto de oclusión de vasos de pequeño calibre por infiltración tumoral, con resultado de múltiples infartos. Las manifestaciones neurológicas suelen ser la forma de presentación clínica inicial, caracterizadas por un amplio espectro que incluye encefalopatía subaguda, deterioro cognitivo, delirio, afasia, hemiparesia, trastornos visuales, paraplejia, parestesias y compromiso de pares craneanos. Los estudios de RNM evidencian alteraciones indistinguibles de las vasculitis del SNC. La linfomatosis intravascular debe considerarse en el diagnóstico diferencial de las demencias rápidamente progresivas y en los cuadros de multi infarto cerebral de pequeño vaso de etiología inusual y recurrente. Se presenta el caso de un paciente con demencia rápidamente progresiva, con manifestaciones neurológicas de deterioro cognitivo y enfermedad sistémica. El compromiso del SNC se expresó como encefalopatía subaguda con confusión, agitación, y crisis convulsivas, trastornos motores con reflejo cutáneo plantar extensor bilateral y compromiso cerebeloso, de curso clínico sintomático progresivo, con baja de peso y fiebre fluctuante. El paciente fue sometido a tratamiento con metilprednisolona a pesar del cual falleció. El estudio necrópsico demostró alteraciones por Linfoma no Hodgkin difuso de células B, variante intra vascular, con compromiso del cerebro, cerebelo, glándulas suprarrenales, páncreas, miocardio, tiroides, pulmón riñón e hígado...

[Revision and classification of persian patient's primary cutaneous lymphoma according to WHO-EORTC classification in Razi hospital, Tehran: a brief report]

Primary cutaneous lymphoma is separated into two groups, cutaneous lymphoma [CTCL] and cutaneous B-CELL Lymphoma [CBCL]. This study was performed to classify histopathologically and define demographic features of Persian patients with primary cutaneous lymphoma. Twenty hundred and thirty four patients with primary cutaneous lymphoma who have been referred to Razi Hospital in Tehran, Iran during at four year period [October 2005 to October 2009], were investigated in this study. The data were gathered by reviewing their medical records and examined again extant evidence and histology slides. Among 234 Patients, the only clinical feature that could be assessed was itching. The most common involves location of disease was generalized. 189 [80.77%] cases of the patients had CTCL and 45 [19.23%] cases of the patients had CBCL. Age of patients with two lymphoma type were similar at the this time of diagnosis. Sex supremacy was with men in two groups. This study provides histopathologic classification and demographic data

Linfoma esofágico primário / Primary esophageal lymphoma

We describe the case of a 54 year old woman seen with an esophageal mass diagnosed as a primary esophageal lymphoma. The main symptom was dysphagia of seven months duration. The treatment consisted in resection of the tumor, and reconstruction of the defect with a reversed pleural flap, followed by a chemotherapy regimen that consisted of five drugs, cyclophosphamid, prednisone, doxorubicin, rituximab and vincristine (R-CHOP). The patient developed an esophageal pleural fistula treated with pleural drainage and irrigation that closed in 45 days. Two and one half years later she is doing well and disease free.

A Case of B-cell Lymphoma, Unclassifiable, with Features Intermediate between Diffuse Large B-cell Lymphoma and Burkitt Lymphoma in a Korean Child

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL) (intermediate DLBCL/BL), is a heterogeneous group with some features resembling DLBCL and others resembling BL. Here, we report a case of intermediate DLBCL/BL in a Korean child. A 2-yr-old male was admitted for evaluation and management of left hip pain. Immunohistochemistry of a biopsy of the femur neck revealed tumor cells positive for CD20, CD10, BCL2, BCL6, and Ki67. A bone marrow (BM) aspirate smear revealed that 49.3% of all nucleated cells were abnormal lymphoid cells, composed of large- and medium-sized cells. Immunophenotyping of the neoplastic cells revealed positivity for CD19, CD10, CD20, and sIg lambda and negativity for CD34, Tdt, and myeloperoxidase (MPO). Cytogenetic and FISH analyses showed a complex karyotype, including t(8;14)(q24.1;q32) and IGH-MYC fusion. Intensive chemotherapy was initiated, including prednisone, vincristine, L-asparaginase, daunorubicin, and central nervous system prophylaxis with intrathecal methotrexate (MTX) and cytarabine. One month after the initial diagnosis, BM examination revealed the persistent of abnormal lymphoid cells; cerebrospinal fluid cytology, including cytospin, showed atypical lymphoid cells. The patient was treated again with cyclophosphamide, vincristine, prednisone, adriamycin, MTX, and intrathecal MTX and cytarabine. The patient died of sepsis 5 months after the second round of chemotherapy.

Apport de l'immunohistochimie dans le diagnostic des lymphomes B agressifs chez les patients infectes ou non par le VIH en Republique democratique du Congo

Objectif. Determiner la contribution potentielle des techniques immunohistochimiques au diagnostic; et dans la prise en charge therapeutique des lymphomes-B agressifs chez des patients infectes ou non par le VIH; en R D Congo; dans le but de vulgarisation de cette approche; complementaire a l'etude morphologique; et indispensable pour le sous-typage de lymphomes; en particulier; de lymphomes-B agressifs. Methodes. Etude transversale et retrospective de 101 blocs de paraffine portant le diagnostic de lymphome et analyses entre 2005 et 2010; par des techniques morphologiques et immunohistochimiques; dans six laboratoires specialises de notre pays. Resultats. Les 81 blocs retenus; etaient tous CD20 positifs et CD3 negatifs; mais le profil etait variable pour d'autres marqueurs etudies. Le lymphome de Burkitt a ete identifie sur 40 blocs; incluant 7 sujets VIH+ (17;5); les lymphomes B diffus a grandes cellules; sur 35; avec 11; chez les VIH+ (31;4).Six lymphomes de forme intermediaire; dont 3; chez des sujets VIH+ (50). Conclusion. Cette etude a permis de classifier les lymphomes-B agressifs dans notre contexte; grace a l'immunohistochimie; justifiant le recours aux anticorps anti-CD20 pour leur traitement

Primary cardiac diffuse large B-cell lymphoma with activated B-cell-like phenotype.

Primary cardiac lymphoma (PCL) is a rare and fatal disorder. It may often mimic other common cardiac tumors like cardiac myxoma because of similarities in the clinical presentation. We report a case of PCL of diffuse large B-cell type, in a 38-year-old, immunocompetent male who presented with superior vena cava syndrome that was excised as a myxoma. Histology revealed a large cell population diffusely and strongly expressing CD45, CD20, MUM1/IRF4 and FOXP1 hinting at an activated B-cell (ABC)-like phenotype. After four cycles of Rituximab with CHOP (cyclophosphamide, hydroxydaunorubicin, Oncovin, and prednisolone) the tumor regressed completely but the patient had a relapse and subsequently succumbed to the disease confirming the aggressive nature. The aggressive behavior of PCL may be possibly linked to its ABC-like origin.

Bilateral primary intraocular lymphoma

To report a case of bilateral primary intraocular lymphoma. A 33-year-old man presented with bilateral blurred vision since two years ago. Examination revealed large keratic precipitates, anterior chamber reaction, posterior subcapsular cataracts, and vitreous infiltration. After a short trial of topical and periocular steroids, diagnostic 25-gauge pars plana vitrectomy was performed and cytologic evaluation of the aspirate confirmed a diagnosis of intraocular lymphoma. The patient was subsequently managed with intravitreal methotrexate in both eyes and responded favorably. Central nervous system workup for lymphoma was negative. Primary intraocular lymphoma should be considered in young adults suffering from chronic recalcitrant panuveitis

Burkitt's lymphoma: clinic progression and prognosis. Two different cases reports in young patients / Linfoma de Burkitt: evolução clínica e prognóstico. Relato de dois casos diferentes em pacientes jovens

Purpose: Burkitt's lymphoma is one of the fastest growing malignancies in the pediatric population. It is a high-grade B-cell non-Hodgkin's lymphoma with endemic, sporadic and human immunodeficiency-associated subtypes. The African, or endemic, variant usually involves the maxilla and other facial bones, while head and neck manifestations in sporadic Burkitt's lymphoma are rare. Case description: Two cases of oral manifestations of Burkitt's lymphoma are described on the right jaw in young patients, which had different clinical evolution and prognosis.

Objetivo: O linfoma de Burkitt é uma das mais importantes neoplasias de crescimento rápido na população pediatra. É um linfoma Não-Hodgkin primário de células B subdividido em endêmico, esporádico ou associado a imunodeficiência humana. O tipo africano (endêmico), quando em cabeça e pescoço, envolve geralmente os maxilares e outros ossos faciais, enquanto que o envolvimento facial da forma esporádica é bem raro. Descrição do caso: São descritos dois casos intra-bucais de linfomas de Burkitt envolvendo maxila direita em pacientes jovens, os quais apresentaram evolução clínica e prognósticos diferentes.

Linfoma B intravascular: comprometimento bilateral da suprarrenal em paciente adulta jovem / Intravascular large B cell lymphoma: impairment of bilateral adrenal in young woman

O linfoma B intravascular de grandes células (IVLBCL) é uma doença rara caracterizada pela proliferação neoplásica de células linfoides no interior de capilares. Relatamos um caso de IVLBCL em paciente jovem do sexo feminino com comprometimento bilateral da glândula suprarrenal. O exame imuno-histoquímico confirmou o IVLBCL. A afinidade entre órgãos endócrinos e células linfoides é uma hipótese levantada para explicar a correlação verificada entre o IVLBCL e o comprometimento da glândula suprarrenal. Além disso, a associação entre o IVLBCL e a sintomatologia descrita parece refletir um padrão que poderá auxiliar em um diagnóstico mais eficaz.

The Intravascular Large B-cell Lymphoma (IVLBCL) is a rare disease characterized by neoplastic proliferation of lymphoid cells within capillaries. We report a case of IVLBCL in a young female patient with bilateral involvement of the adrenal gland. Immunohistochemical examination confirmed IVLBCL. The relationship between endocrine organs and lymphoid cells is a hypothesis to explain the correlation observed between IVLBCL and the involvement of the adrenal gland. Moreover, the association between IVLBCL and the described symptomatology seems to reflect a pattern that may assist in a more effective diagnosis.