Pediatric Anaplastic Large Cell Lymphoma—A Review

Tole, Soumitra; Wheaton, Laura; Alexander, Sarah

doi:10.17925/ohr.2018.14.1.21

Cited by 7 publications

(5 citation statements)

References 58 publications

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“…Anaplastic large cell lymphoma is a type of non-Hodgkin’s lymphoma (NHL) and constitutes 10% to 15% of paediatric NHL. The classical presentation of ALCL includes lymphadenopathy and fever with constitutional symptoms; extranodal manifestations including involvement of skin, lungs and bones are common in ALCL [ 5 ]. The constitutive expression and activation of oncogenic ALK fusion proteins, due to rearrangements of the ALK gene with the nucleophosmin gene (NPM1) leading to t (2:5) translocation, is seen in the majority of ALCL cases or, less frequently, with gene partners other than NPM1 [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

Malignancy-Associated Secondary Hemophagocytic Lymphohistiocytosis Mimicking an Infection: A Case Report and Review of the Literature

Gopalakrishnan,

Ramanathan,

Jayaraman

et al. 2024

Cureus

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Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening hematological disorder of immune dysregulation associated with significant challenges in diagnosis and management. Described as primary HLH secondary to genetic defects or more commonly secondary to infections, it can also occur secondary to malignancy, i.e., malignancy-associated hemophagocytic lymphohistiocytosis (M-HLH). A five-year-old male child presented with left cervical adenopathy and a high-spiking fever for two weeks. He had pallor, anasarca, multiple enlarged and matted cervical lymph nodes, respiratory distress, and hepatomegaly. He had continuous high-grade fever spikes (maximum 105 °F), not touching baseline despite broad-spectrum antibiotics. The CBC revealed anemia with thrombocytopenia. Liver function tests showed mild transaminitis and hypoalbuminemia. The HLH workup showed elevated ferritin, low fibrinogen, and elevated triglycerides. Lymph node biopsy showed intermediate to large atypical monomorphic lymphocyte cells with ALK, CD30, CD5, CD3, CD45, and BCL-2 (weak positive) positivity and Ki-67-95%, suggestive of anaplastic large cell lymphoma (ALCL). The bone marrow aspiration showed reactive marrow with hemophagocytosis. The patient was started on dexamethasone and chemotherapy per the Children's Oncology Group's (COG) ALCL protocol. He showed remarkable clinical improvement and went into remission after the induction phase. Malignancy associated with HLH can mimic infection, as in our patient with high-spiking fever, consolidation, and mediastinal adenopathy. A high index of suspicion is necessary to arrive at an appropriate, early diagnosis, and workup for malignancy is to be considered when an infectious etiology is not identified after thorough evaluation.

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Section: Discussionmentioning

confidence: 99%

Malignancy-Associated Secondary Hemophagocytic Lymphohistiocytosis Mimicking an Infection: A Case Report and Review of the Literature

Gopalakrishnan,

Ramanathan,

Jayaraman

et al. 2024

Cureus

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“…15,20 In children, more than 95% of ALCL is ALK+, predominantly due to a translocation NPM-ALK fusion t(2;5)(p23;q35) making it one of the ideal targets. [21][22][23] Crizotinib (CRZ), a first-generation ALK inhibitor, studied in ALK+ pediatric ALCL and in adults with NSCLC showed good responses and prolonged survival. [24][25][26][27] However, studies have demonstrated low CSF concentrations of CRZ during systemic chemotherapy.…”

Section: Discussionmentioning

confidence: 99%

Central nervous system relapse in a child with anaplastic large cell lymphoma: potential for new therapeutic strategies

et al. 2021

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Background: Central nervous system (CNS) relapse is rare in childhood anaplastic large cell lymphoma (ALCL) and is associated with a poor prognosis. Case:We describe an 8-year-old boy with ALCL who developed an early CNS relapse without initial CNS disease. Despite aggressive medical management, the patient's neurological status deteriorated rapidly and he died shortly after. Conclusion:Optimal treatment for children with relapsed ALCL involving the CNS remains unclear. Novel agents, including ALK inhibitors, that have CNS-penetration might be helpful and pediatric studies are warranted.

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“…They act by inhibiting the permanently activated abnormal ALK protein, blocking downstream signaling pathways involved in tumoral growth. Over the years, the use of ALK inhibitors has been studied and implemented in particular in ALK-positive non-small-cell lung cancer (NSCLC), but represents a therapeutic option in a series of other tumors such as pediatric ALCL, inflammatory myofibroblastic tumor (IMT) and neuroblastoma, which can express different types of ALK oncogenic mutations [21]. Table 2 lists the main ALK inhibitors currently approved for use in clinical practice.…”

Section: Alk Inhibitorsmentioning

confidence: 99%

Refractory Anaplastic Large Cell Lymphoma Rescued by the Combination of the Second-Generation ALK Inhibitor Brigatinib, High-dose Chemotherapy and Allogeneic Stem Cell Transplantation: A Case Report and Review of the Literature

et al. 2023

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The treatment of pediatric patients with refractory or relapsed anaplastic large cell lymphoma (ALCL) is still a major challenge. In addition to conventional chemotherapy and stem cell transplantation, new therapeutic options such as anti-CD30 drugs and anaplastic lymphoma kinase (ALK) inhibitors have been recently introduced in this setting. Among ALK inhibitors, only the first-generation molecule crizotinib is approved for pediatric use, while second-generation molecules, such as brigatinib, are still under investigation. Here we report the case of a 13-year-old boy diagnosed with stage IV ALCL, refractory to first-line conventional chemotherapy and second-line therapy with the anti CD30 antibody–drug conjugate brentuximab-vedotin, who finally achieved remission after a combination of conventional high-dose chemotherapy and the second-generation ALK inhibitor brigatinib. The latter was chosen for its ability to penetrate through the blood–brain barrier, due to the persistent involvement of the patient’s cerebral nervous system. The remission was then consolidated with an allogeneic hematopoietic stem cell transplantation (HSCT) from an unrelated donor using myeloablative conditioning with total body irradiation. At 24 months after HSCT, the patient is in complete remission, alive and well. An updated review regarding the use of ALK inhibitors in ALCL patients is provided.

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Pediatric Anaplastic Large Cell Lymphoma—A Review

Cited by 7 publications

References 58 publications

Malignancy-Associated Secondary Hemophagocytic Lymphohistiocytosis Mimicking an Infection: A Case Report and Review of the Literature

Malignancy-Associated Secondary Hemophagocytic Lymphohistiocytosis Mimicking an Infection: A Case Report and Review of the Literature

Central nervous system relapse in a child with anaplastic large cell lymphoma: potential for new therapeutic strategies

Refractory Anaplastic Large Cell Lymphoma Rescued by the Combination of the Second-Generation ALK Inhibitor Brigatinib, High-dose Chemotherapy and Allogeneic Stem Cell Transplantation: A Case Report and Review of the Literature

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