Lymphoblastic Lymphoma Presenting Research Articles

Lymphoblastic lymphoma presenting as soft-tissue swelling – A single-center experience

Abstract: BACKGROUND: Lymphoblastic lymphomas (LBL) are a rare, aggressive type of non-Hodgkin lymphomas and constitute 2% of all lymphomas. It is classified as T-lymphoblastic lymphoma (T-LBL) and B lymphoblastic lymphoma (B-LBL) and resembles acute lymphoblastic leukemia (ALL), with no or limited bone-marrow involvement. LBL rarely present as soft-tissue swellings. Soft-tissue involvement is more common in B-LBL than in T-LBL. They occur predominantly in adolescents and young adults and have a prognosis similar to ALL. OBJECTIVE: p>The aim was to study the clinical characteristics, treatment response, and survival of patients with lymphoblastic lymphoma presenting with soft-tissue swelling. SETTINGS AND DESIGN: This was a retrospective study of eight adolescent and adult patients with lymphoblastic lymphoma presenting with soft-tissue swelling conducted in the Department of Medical Oncology at a tertiary cancer center in India. STATISTICAL ANALYSIS: The baseline patient characteristics, treatment details, and response assessment were analyzed using descriptive statistics. Overall survival (OS) and progression-free survival were obtained by the Kaplan–Meier method, using the SPSS version. 11. MATERIALS AND METHODS: We present the clinical features, imaging, diagnosis, treatment, and outcome of eight cases of lymphoblastic lymphoma presenting with soft-tissue swellings. Patients above 14 years of age treated over 12 years were included in the study. RESULTS: There were five males and three females. The median age at diagnosis was 24 years. Common presenting complaints were swelling, pain, and paraparesis. The sites of involvement were the epidural mass, thigh, calf, breast, and anterior chest wall. The diagnosis was confirmed by immunohistochemistry. Seven patients were diagnosed with B-LBL and one with T-LBL. They were treated with intensive chemotherapy (six patients with Berlin–Frankfurt–Munster protocol and two with hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone). Three patients received consolidation radiotherapy and one received palliative radiotherapy. Seven patients attained remission after induction chemotherapy and four of them are currently alive at a median follow-up of 48 months. The 2-year OS was 71.4%. CONCLUSION: Even though rare, lymphoblastic lymphoma should be considered a differential diagnosis in patients presenting with soft-tissue swelling and should be managed with systemic chemotherapy similar to ALL.

Precursor B-cell lymphoblastic lymphoma presenting as a primary solitary skull bone tumor

1Division of Neurosurgery, Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan 2Post Baccalaureate Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan 3School of Medicine. College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan Address for correspondence: Dr. Ann-Shung Lieu, Division of Neurosurgery, Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan, No. 100, Tzyou 1st Road, Kaohsiung 80708, Taiwan. E-mail: [email protected]. Data availability statement: Data sharing not applicable to this article as no datasets were generated or analyzed during the current study. Financial support and sponsorship: Nil. Conflicts of interest: There are no conflicts of interest. Submitted: 31-Aug-2022, Accepted: 01-Sep-2022 This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

A Rare Case of Biliary Duct Lymphoblastic Lymphoma

Background: Lymphoblastic lymphoma (LBL) presenting with obstructive jaundice cases are rare. It is rarely considered in the differential diagnosis of patients with obstructive jaundice. Case description: A young woman complained of jaundice, tea-colored urine, pale stools, and lost weight. The abdominal examination showed mild tenderness in the epigastric area. Laboratory showed elevation of liver function, bilirubin, and alkaline phosphatase. The radiological examination both in abdominal ultrasound and magnetic resonance cholangio-pancreatography suggested a possible differential diagnosis between of lymphoma and cholangiocarcinoma. She underwent surgery for open biopsy from biliary duct and showed lymphoblastic lymphoma. She was received combination of chemotherapy. She had significant improvement of her quality of life. Conclusion: The diagnosis of biliary duct lymphoblastic lymphoma is essential but can be very challenging. Clinical diagnosis is based on symptoms and signs, while laboratory testing is non-specific and occasionally misleading. However, direct imaging and biopsy guidance is highly recommended.

Presentation of Acute Lymphoblastic Lymphoma and Colorectal Carcinoma in the Context of Constitutional Mismatch Repair Deficiency Syndrome: A Case Report with Literature Review.

Constitutional mismatch repair deficiency (CMMRD) is a rare autosomal recessive disease carrying an increased risk of cancers (paediatric tumours of central nervous system, haematolymphoid malignancies along with gastrointestinal (GI) cancer(s), which are usually seen in the second and third decades), leading to syndromic presentation. Causal mutations are detected in DNA mismatch repair (MMR) genes, including MLH1, PMS2, MSH2 and MSH6 that are also known for their established role in Lynch syndrome. We describe a case of CMMRD with an earlier (first decade of life) presentation of mediastinal acute lymphoblastic lymphoma and colorectal malignancy. A 5-year-old boy presented with respiratory complaints, bilateral cervical lymphadenopathy, multiple café-au-lait macules (CALMs) on the lower back and history of parental consanguinity with the death of three sisters due to brain tumour within 6 months of diagnosis. Computerised tomographic scan chest revealed a huge mediastinal mass. The patient underwent a trucut biopsy of the mass. The results were significant for a pre-T-cell acute lymphoblastic lymphoma. Suspicion of CMMRD was raised based on a combination of factors described above. A panel of MMR proteins was applied on the biopsy tissue that revealed loss of nuclear expression of MLH1 and PMS2 immunostaining in tumour cells with positive external controls. While on maintenance therapy for lymphoma, about a year later, the patient developed subacute intestinal obstruction due to a stenosing polypoidal circumferential tumour in the mid-sigmoid colon found on flexible sigmoidoscopy that was followed by endoscopic biopsies and insertion of a fully covered self-expanding metallic adult biliary stent with a diameter of 10 mm and length of 6 cm leading to immediate relief of obstruction. Biopsies revealed adenocarcinoma with neuroendocrine differentiation. Metastatic tumour deposits were seen in the omentum, anterior abdominal wall and the left peritoneal wall. Earlier (first decade) presentation of GI malignancy warrants that an earlier screening through radiological scans for any possible tumours and MMR protein expression analysis (loss in tumour plus normal non-tumour cells) are essential in patients having CALMs and family history of paediatric tumours.

Hyperoeosinophilia at diagnosis in adolescent acute lymphoblastic leukaemia/lymphoma: A case report and review of the literature

Acute lymphoblastic leukaemia (ALL) or lymphoblastic lymphoma (LBL) presenting as peripheral blood hyperoeosinophilia is a sporadic disorder with fewer than fifty cases described since 1973, and the cumulative incidence is

Adult T-type lymphoblastic lymphoma presenting as hypercalcemic crisis and aplastic anemia: a case report

BackgroundHypercalcemia and aplastic anemia are two uncommon presentations of non-Hodgkin lymphoma that potentially worsen the disease prognosis. Although hypercalcemia has been reported in the B-cell subtypes and some T-cell subtypes of non-Hodgkin lymphoma, it has not been described in T-cell lymphoblastic lymphoma. The same applies to aplastic anemia, which is also not described in T-type lymphomas.Case presentationWe report a case of a 52-year-old Cameroonian man with acute kidney injury who presented with confusion, abdominal pain, constipation, polyuria, polydipsia, calciphylaxis, enlarged lymph nodes, tachycardia, and a blood pressure of 170/88 mmHg. Laboratory investigations revealed hypercalcemia (total/ionized 199.5/101.75 mg/L), normal serum phosphorus (40.20 mg/L), and a low intact parathyroid hormone (9.70 pg/ml). Complete blood count revealed pancytopenia. Peripheral blood smear confirmed thrombocytopenia but showed neither blasts nor flower cells. Bone marrow aspirate revealed hypocellularity with no blasts or fibrosis. Lymph node biopsy was suggestive of T-cell precursor lymphoma. T-lymphoblastic lymphoma presenting with hypercalcemic crisis and aplastic anemia was diagnosed, and the patient received the cyclophosphamide-doxorubicin-vincristine-prednisone protocol of chemotherapy together with filgrastim and whole-blood transfusion for aplastic anemia. The short-term outcome was fatal, however.ConclusionsSevere hypercalcemia and aplastic anemia are potential paraneoplastic syndromes of adult T-type lymphoblastic lymphoma, with fatal short-term outcome.

Pericardial effusion and constriction as an initial presentation of acute t-cell lymphoblastic lymphoma

Acute T-cell lymphoblastic lymphoma is a rare subtype of non-Hodgkin’s lymphomaaccounting for 2% of adult non-Hodgkin’s lymphomas. Cardiac involvement in non-Hodgkin’slymphoma is usually a late manifestation, and pericardial effusion as an initial presentationis rare. We report a case of acute T-cell lymphoblastic lymphoma in a patient who initiallypresented with pericardial effusion. Diagnosis was difficult and challenging, as an infectiousetiology was first suspected. The patient presented with pericardial effusion without othercommon clinical manifestations of lymphoma, such as fever, night sweats, and weight loss. Hewas treated for constrictive pericarditis due to having positive Coxsackie B serology but hadno improvement with treatment. The pathology results of the pericardium after pericardiectomyreported fibrosis with a reactive lymphohistiocyte infiltrate. After CT scan revealed a largemediastinal mass, a lymph node biopsy was performed, and T-cell lymphoblastic lymphomawas confirmed. A detailed evaluation to search for occult malignancy should be considered inpatients who have persistent pericarditis that is unresponsive to anti-inflammatory therapy andin those who present with a new, large pericardial effusion or cardiac tamponade.

T-Cell Acute Lymphoblastic Lymphoma Presenting as Dissecting Cellulitis and Cavernous Sinus Syndrome

T-Cell Acute Lymphoblastic Lymphoma Presenting as Dissecting Cellulitis and Cavernous Sinus Syndrome

Lymphoblastic Lymphoma Presenting as a Lymphocyte-Rich Thymoma Recurrence

Lymphoblastic Lymphoma Presenting as a Lymphocyte-Rich Thymoma Recurrence

A unique bone marrow lymphoma patient presenting with an isolated mass: A case report.

Bone marrow lymphoma with the onset of an isolated mass in the bone marrow is extremely rare. The present case report described a unique case of B cell lymphoblastic lymphoma (LBL) presenting with an isolated mass in the bone marrow cavity, without any organopathy or lymphadenopathy. An isolated mass in bone marrow is a rare primary manifestation of LBL. The patient in the present case report presented with pain in the right elbow, a fever, pancytopenia and splenomegaly. Additionally, no abnormality was determined in the lymph nodes, the bone marrow karyotype or a computed tomography scan of the humerus. Positron emission tomography (PET) examination revealed an increased uptake of 18F-fluorodeoxyglucose in right distal humerus. An isolated mass in the bone marrow cavity was removed by surgery. Pathological findings revealed B cell LBL. The patient received an acute lymphocytic leukemia chemotherapy regimen and achieved complete remission. However, 4 months following the initial diagnosis, the patient succumbed due to a relapse. The present case highlighted the importance of PET examination and biopsy, and the requirement to identify appropriate treatments for LBL. Additionally, it is important to broaden the differential diagnosis when an isolated mass is identified in the bone marrow cavity.

Aches and Pain in a child- An unusual cause

Precursor B-cell lymphoblastic lymphoma presenting with severe pain and extensive skeletal lesions as an initial presentation is very rare. We report a nine year old boy who presented with lower backache and difficulty in walking of 2 months duration. He neither had lymphadenopathy nor organomegaly. His peripheral blood picture and bone marrow examination were normal. MRI revealed multiple lytic lesions in the spine and PET scan revealed metabolically active lytic lesions in the axial skeletal system and bilateral femurs and humerus. Biopsy of the vertebra revealed the diagnosis of precursor B cell lymphoma. A diagnosis of malignancy should never be missed in a patient with musculoskeletal pain, having ruled out infectious and traumatic etiologies especially when they present with several skeletal radiolucencies.

Acute B-Cell Lymphoblastic Leukemia/Acute B-Cell Lymphoblastic Lymphoma Presenting as Bilateral Breast Masses

Acute B-Cell Lymphoblastic Leukemia/Acute B-Cell Lymphoblastic Lymphoma Presenting as Bilateral Breast Masses

Disseminated Presentation of Primary Lymphoblastic Lymphoma of the Bone in a Pediatric Patient

Primary non-Hodgkin’s lymphoma of the bone (PLB) is extremely rare in the pediatric population with less than 100 cases reported in the English literature. Most commonly, patients present with atraumatic bone pain and grossly normal radiographic findings. PLB is in the histopathological class of “small round cell tumors of bone”, as with most common bone tumors. The diagnosis is confirmed by immunohistochemical or flow cytometry based detection of tumor-specific proteins. We present a case of stage IV PLB of B-lymphoblastic type with an excellent response to chemotherapy to increase awareness among general pediatricians and pathologists about the importance of making the correct diagnosis, given the excellent prognosis for this disease.

Raccoon eyes as presentation of lymphoblastic lymphoma in a child

Raccoon eyes as presentation of lymphoblastic lymphoma in a child

Pediatric precursor B-cell lymphoblastic lymphoma presenting with extensive skeletal lesions

Lymphoblastic lymphoma (LL) of the B cell type is uncommon, and extranodal presentation is even rarer. It is difficult to suspect this diagnosis without clinically obvious lymph nodal mass or bone marrow involvement. A 3-year-old girl presented with progressive pain and swelling of the right knee joint of 3 months duration. Radiograph revealed expansile lytic lesion at the supracondylar area of the right femur; with pathological fracture and multiple lytic areas in both femora. She neither had lymphadenopathy nor organomegaly. Her blood counts, peripheral smear examination and bone marrow examination were normal. Right supracondylar biopsy revealed diagnosis of a precursor B cell LL. Computerized tomography scan revealed a hypodense, poorly enhancing lesion in the left adnexal region. Although rare, precursor B-cell LL may present with extensive bone lesions. Early and accurate diagnosis of this entity is very important due to its high cure rates.

Acute Kidney Injury and Bilateral Symmetrical Enlargement of the Kidneys as First Presentation of B-Cell Lymphoblastic Lymphoma

Lymphoblastic lymphoma is an uncommon subtype of lymphoid neoplasm in adults. Acute kidney injury at initial presentation due to lymphoblastic lymphoma infiltration of the kidneys has rarely been described. We report a 19-year-old woman who presented with acute kidney injury due to massive lymphomatous infiltration of the kidneys. The diagnosis of B-cell lymphoblastic lymphoma was established by immunohistochemical study of the biopsied kidney. The patient had an excellent response to the VDCLP protocol (vincristine, daunomycin, cyclophosphamide, asparaginase, and dexamethasone) with sustained remission. We recommend that lymphomatous infiltration be considered in patients presenting with unexplained acute kidney injury and enlarged kidneys.

Follicular lymphoma transformed to “double‐hit” B lymphoblastic lymphoma presenting in the peritoneal fluid

Lymphomas showing both MYC/8q24 rearrangement and IGH@BCL2/t(14;18)(q32;q21), also referred to as "double-hit" or "dual-hit" lymphomas (DHL) are rare B-cell malignancies with a germinal center B-cell immunophenotype and heterogeneous cytologic and histologic features. Such lymphomas may arise de novo or through transformation of follicular lymphomas and are classified either as "B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL)" (most commonly), DLBCL, or, rarely, as B-lymphoblastic lymphoma. We report a case of B-lymphobastic lymphoma arising through transformation of follicular lymphoma diagnosed on peritoneal fluid cytology, flow cytometry, and cytogenetic studies in a 53-year-old man who presented with abdominal pain, shortness of breath, night sweats, extensive lymphadenopathy, pleural effusion, and ascites. Cytologic examination of the ascitic fluid showed two distinct populations of neoplastic lymphoid cells, a predominant population of larger cells with fine powdery ("blastic") chromatin, visible to prominent nucleoli and occasional small cytoplasmic vacuoles and a less numerous population of smaller cells with centrocytic morphology. Flow cytometry also showed two distinct monotypic B-cell populations, both expressing CD10, and TdT-positivity was demonstrated immunohistochemically. Fluorescence in situ hybridization (FISH) demonstrated both MYC rearrangement and IGH/BCL2 gene fusion and cytogenetic analysis showed a complex karyotype including both t(14;18)(q32;q21) and t(8;22)(q24.1;q11.2). Since DHL pursue an aggressive clinical course, respond poorly to therapy, and have a poor outcome, it is important to suspect the diagnosis when encountering neoplastic lymphoid cells that are difficult to classify in effusion cytology specimens and to order the appropriate immunophenotyping and cytogenetic studies.

Precursor B cell lymphoblastic lymphoma presenting as a solitary bone tumor: a case report and review of the literature

Precursor B cell lymphoblastic lymphoma (B-LBL) is quite uncommon and it usually manifests as an extranodal disease. Although B-LBL may present with bone involvement, it is a very rare manifestation of B-LBL as a primary solitary bone tumor. Here, we report a case of precursor B-LBL presenting with solitary bone tumor and a review of a total of seven adult patients reported previously in the literature. We described demographic and clinical characteristics of these patients with unique presentation and discussed treatment options. Unlike previous reports except one case, our patient underwent allogeneic stem cell transplantation (allo-SCT) due to refractory disease. She is alive without evidence of disease by the post-transplant 12th month. B-LBL has an aggressive clinical course in adult patients and allo-SCT may be the best treatment option.

T-Cell Lymphoblastic Lymphoma Presenting as Back Pain in a Crohnʼs Disease Patient Taking Adalimumab (Humira)

T-Cell Lymphoblastic Lymphoma Presenting as Back Pain in a Crohnʼs Disease Patient Taking Adalimumab (Humira)

B Lymphoblastic Lymphoma Presenting as a Tumor of the Nasopharynx in an Adult Patient

In adults, non-Hodgkin's lymphoma (NHL) is the second most common neoplasm found in the head and neck region after squamous cell carcinoma. Within this region, primary NHL of the nasopharynx is rare. We report the case of a 28-year-old male diagnosed with a B lymphoblastic lymphoma (CD20-; CD79a+; CD3-; CD10+; PAX5+, CyclinD1-; TdT+) of the nasopharynx extending to the deep and superficial structures of the right hemiface, to the skull base with an intracranial component and a small but detectable bone marrow involvement, who was started on chemotherapy with a complete response. To the best of our knowledge, this is the first case of a primary nasopharynx B-LBL in an adult patient with such aggressive regional spread to be reported in the literature.