Fatigue and sporadic fever

This patient's findings are consistent with a diagnosis of malignant mantle cell lymphoma (MCL).

MCL is a rare and aggressive form of non-Hodgkin lymphoma that accounts for approximately 5%-7% of all lymphomas. MCL has a characteristic immunophenotype (ie, CD5+, CD10−, Bcl-2+, Bcl-6−, CD20+), with the t(11;14)(q13;q32) chromosomal translocation, and expression of cyclin D1. The median age at diagnosis is between 60 and 70 years. Approximately 70% of all cases occur in men. 

The clinical presentation of MCL can vary. Patients may have asymptomatic monoclonal MCL type lymphocytosis or nonbulky nodal/extra nodal disease with minimal symptoms, or they may present with significant symptoms, progressive generalized lymphadenopathy, cytopenia, splenomegaly, and extranodal disease, including gastrointestinal involvement (lymphomatous polyposis), kidney involvement, involvement of other organs, or, rarely, central nervous system involvement. Disease involving multiple lymph nodes and other sites of the body is seen in most patients. Approximately 70% of patients present with stage IV disease requiring systemic treatment.

According to 2022 guidelines from the National Comprehensive Cancer Network (NCCN), essential components in the workup for MCL include:

•    Physical examination, with attention to node-bearing areas, including Waldeyer ring, and to size of liver and spleen
•    Assessment of performance status and B symptoms (ie, fever > 100.4°F [may be sporadic], drenching night sweats, unintentional weight loss of > 10% of body weight over 6 months or less)
•    CBC with differential
•    Comprehensive metabolic panel
•    Serum lactate dehydrogenase (LDH) level (an important prognostic marker)
•    PET/CT scan (including neck)
•    Hepatitis B testing if treatment with rituximab is being contemplated
•    Echocardiogram or multigated acquisition (MUGA) scan if anthracycline or anthracenedione-based regimen is indicated
•    Pregnancy testing in women of childbearing age (if chemotherapy or radiation therapy is planned) 

Additional testing may be indicated in specific circumstances, such as colonoscopy/endoscopy. 

MCL remains challenging to treat. While 50%-90% of patients with MCL respond to combination chemotherapy, only 30% achieve a complete response. Median time to treatment failure is < 18 months. 

When selecting systemic treatment for patients with MCL, clinicians should consider the availability of clinical trials for subsets of patients, eligibility for stem cell transplant (SCT), high-risk status (ie, blastoid MCL, high Ki-67% > 30%, or central nervous system involvement), age, and performance status. The addition of radiation to chemotherapy may be beneficial for patients with limited-stage, nonbulky disease, although this has not been confirmed in large, randomized studies. Outside of clinical trials, the usual approach for frontline treatment of MCL is chemoimmunotherapy with/without autologous SCT and with/without maintenance therapy.

Available options for primary MCL therapy in patients who require systemic therapy include: 

•    Single alkylating agents
•    CVP (cyclophosphamide, vincristine, prednisone)
•    CHOP (cyclophosphamide, doxorubicin [hydroxydaunorubicin], vincristine [Oncovin], prednisone)
•    Hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone) with or without rituximab
•    R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)
•    Lenalidomide plus rituximab
•    Hyper-CVAD with autologous SCT

Options for relapsed or refractory MCL include:

•    R-hyper-CVAD
•    Hyper-CVAD with or without rituximab followed by autologous SCT
•    Nucleoside analogues and combinations
•    Salvage chemotherapy combinations followed by autologous SCT
•    Bortezomib 
•    Lenalidomide 
•    Ibrutinib 
•    Radioimmunotherapy
•    Rituximab
•    Rituximab and thalidomide combination
•    Acalabrutinib 
•    High-dose chemotherapy with autologous bone marrow or SCT
•    Brexucabtagene autoleucel 

Timothy J. Voorhees, MD, MSCR, Assistant Professor of Internal Medicine - Clinical, Division of Hematology, The Ohio State University James Comprehensive Cancer Center, Columbus, OH.

Timothy J. Voorhees, MD, MSCR, has disclosed the following relevant financial relationships:
Received research grant from: AstraZeneca; Morphosys; Incyte; Recordati.

Image Quizzes are fictional or fictionalized clinical scenarios intended to provide evidence-based educational takeaways.

This patient's findings are consistent with a diagnosis of malignant mantle cell lymphoma (MCL).

MCL is a rare and aggressive form of non-Hodgkin lymphoma that accounts for approximately 5%-7% of all lymphomas. MCL has a characteristic immunophenotype (ie, CD5+, CD10−, Bcl-2+, Bcl-6−, CD20+), with the t(11;14)(q13;q32) chromosomal translocation, and expression of cyclin D1. The median age at diagnosis is between 60 and 70 years. Approximately 70% of all cases occur in men. 

The clinical presentation of MCL can vary. Patients may have asymptomatic monoclonal MCL type lymphocytosis or nonbulky nodal/extra nodal disease with minimal symptoms, or they may present with significant symptoms, progressive generalized lymphadenopathy, cytopenia, splenomegaly, and extranodal disease, including gastrointestinal involvement (lymphomatous polyposis), kidney involvement, involvement of other organs, or, rarely, central nervous system involvement. Disease involving multiple lymph nodes and other sites of the body is seen in most patients. Approximately 70% of patients present with stage IV disease requiring systemic treatment.

According to 2022 guidelines from the National Comprehensive Cancer Network (NCCN), essential components in the workup for MCL include:

•    Physical examination, with attention to node-bearing areas, including Waldeyer ring, and to size of liver and spleen
•    Assessment of performance status and B symptoms (ie, fever > 100.4°F [may be sporadic], drenching night sweats, unintentional weight loss of > 10% of body weight over 6 months or less)
•    CBC with differential
•    Comprehensive metabolic panel
•    Serum lactate dehydrogenase (LDH) level (an important prognostic marker)
•    PET/CT scan (including neck)
•    Hepatitis B testing if treatment with rituximab is being contemplated
•    Echocardiogram or multigated acquisition (MUGA) scan if anthracycline or anthracenedione-based regimen is indicated
•    Pregnancy testing in women of childbearing age (if chemotherapy or radiation therapy is planned) 

Additional testing may be indicated in specific circumstances, such as colonoscopy/endoscopy. 

MCL remains challenging to treat. While 50%-90% of patients with MCL respond to combination chemotherapy, only 30% achieve a complete response. Median time to treatment failure is < 18 months. 

When selecting systemic treatment for patients with MCL, clinicians should consider the availability of clinical trials for subsets of patients, eligibility for stem cell transplant (SCT), high-risk status (ie, blastoid MCL, high Ki-67% > 30%, or central nervous system involvement), age, and performance status. The addition of radiation to chemotherapy may be beneficial for patients with limited-stage, nonbulky disease, although this has not been confirmed in large, randomized studies. Outside of clinical trials, the usual approach for frontline treatment of MCL is chemoimmunotherapy with/without autologous SCT and with/without maintenance therapy.

Available options for primary MCL therapy in patients who require systemic therapy include: 

•    Single alkylating agents
•    CVP (cyclophosphamide, vincristine, prednisone)
•    CHOP (cyclophosphamide, doxorubicin [hydroxydaunorubicin], vincristine [Oncovin], prednisone)
•    Hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone) with or without rituximab
•    R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)
•    Lenalidomide plus rituximab
•    Hyper-CVAD with autologous SCT

Options for relapsed or refractory MCL include:

•    R-hyper-CVAD
•    Hyper-CVAD with or without rituximab followed by autologous SCT
•    Nucleoside analogues and combinations
•    Salvage chemotherapy combinations followed by autologous SCT
•    Bortezomib 
•    Lenalidomide 
•    Ibrutinib 
•    Radioimmunotherapy
•    Rituximab
•    Rituximab and thalidomide combination
•    Acalabrutinib 
•    High-dose chemotherapy with autologous bone marrow or SCT
•    Brexucabtagene autoleucel 

Timothy J. Voorhees, MD, MSCR, Assistant Professor of Internal Medicine - Clinical, Division of Hematology, The Ohio State University James Comprehensive Cancer Center, Columbus, OH.

Timothy J. Voorhees, MD, MSCR, has disclosed the following relevant financial relationships:
Received research grant from: AstraZeneca; Morphosys; Incyte; Recordati.

Image Quizzes are fictional or fictionalized clinical scenarios intended to provide evidence-based educational takeaways.

This patient's findings are consistent with a diagnosis of malignant mantle cell lymphoma (MCL).

MCL is a rare and aggressive form of non-Hodgkin lymphoma that accounts for approximately 5%-7% of all lymphomas. MCL has a characteristic immunophenotype (ie, CD5+, CD10−, Bcl-2+, Bcl-6−, CD20+), with the t(11;14)(q13;q32) chromosomal translocation, and expression of cyclin D1. The median age at diagnosis is between 60 and 70 years. Approximately 70% of all cases occur in men. 

The clinical presentation of MCL can vary. Patients may have asymptomatic monoclonal MCL type lymphocytosis or nonbulky nodal/extra nodal disease with minimal symptoms, or they may present with significant symptoms, progressive generalized lymphadenopathy, cytopenia, splenomegaly, and extranodal disease, including gastrointestinal involvement (lymphomatous polyposis), kidney involvement, involvement of other organs, or, rarely, central nervous system involvement. Disease involving multiple lymph nodes and other sites of the body is seen in most patients. Approximately 70% of patients present with stage IV disease requiring systemic treatment.

According to 2022 guidelines from the National Comprehensive Cancer Network (NCCN), essential components in the workup for MCL include:

•    Physical examination, with attention to node-bearing areas, including Waldeyer ring, and to size of liver and spleen
•    Assessment of performance status and B symptoms (ie, fever > 100.4°F [may be sporadic], drenching night sweats, unintentional weight loss of > 10% of body weight over 6 months or less)
•    CBC with differential
•    Comprehensive metabolic panel
•    Serum lactate dehydrogenase (LDH) level (an important prognostic marker)
•    PET/CT scan (including neck)
•    Hepatitis B testing if treatment with rituximab is being contemplated
•    Echocardiogram or multigated acquisition (MUGA) scan if anthracycline or anthracenedione-based regimen is indicated
•    Pregnancy testing in women of childbearing age (if chemotherapy or radiation therapy is planned) 

Additional testing may be indicated in specific circumstances, such as colonoscopy/endoscopy. 

MCL remains challenging to treat. While 50%-90% of patients with MCL respond to combination chemotherapy, only 30% achieve a complete response. Median time to treatment failure is < 18 months. 

When selecting systemic treatment for patients with MCL, clinicians should consider the availability of clinical trials for subsets of patients, eligibility for stem cell transplant (SCT), high-risk status (ie, blastoid MCL, high Ki-67% > 30%, or central nervous system involvement), age, and performance status. The addition of radiation to chemotherapy may be beneficial for patients with limited-stage, nonbulky disease, although this has not been confirmed in large, randomized studies. Outside of clinical trials, the usual approach for frontline treatment of MCL is chemoimmunotherapy with/without autologous SCT and with/without maintenance therapy.

Available options for primary MCL therapy in patients who require systemic therapy include: 

•    Single alkylating agents
•    CVP (cyclophosphamide, vincristine, prednisone)
•    CHOP (cyclophosphamide, doxorubicin [hydroxydaunorubicin], vincristine [Oncovin], prednisone)
•    Hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone) with or without rituximab
•    R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone)
•    Lenalidomide plus rituximab
•    Hyper-CVAD with autologous SCT

Options for relapsed or refractory MCL include:

•    R-hyper-CVAD
•    Hyper-CVAD with or without rituximab followed by autologous SCT
•    Nucleoside analogues and combinations
•    Salvage chemotherapy combinations followed by autologous SCT
•    Bortezomib 
•    Lenalidomide 
•    Ibrutinib 
•    Radioimmunotherapy
•    Rituximab
•    Rituximab and thalidomide combination
•    Acalabrutinib 
•    High-dose chemotherapy with autologous bone marrow or SCT
•    Brexucabtagene autoleucel 

Timothy J. Voorhees, MD, MSCR, Assistant Professor of Internal Medicine - Clinical, Division of Hematology, The Ohio State University James Comprehensive Cancer Center, Columbus, OH.

Timothy J. Voorhees, MD, MSCR, has disclosed the following relevant financial relationships:
Received research grant from: AstraZeneca; Morphosys; Incyte; Recordati.

Image Quizzes are fictional or fictionalized clinical scenarios intended to provide evidence-based educational takeaways.