ALK-positive large B-cell lymphoma

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2FDA treatments3Active trials8Specialists8Treatment centers1Financial resources

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Overview

ALK-positive large B-cell lymphoma (also called ALK+ LBCL or ALK-positive diffuse large B-cell lymphoma) is a very rare type of non-Hodgkin lymphoma, which is a cancer of the immune system's B cells. In this disease, the cancer cells carry an abnormal activation of a protein called ALK (anaplastic lymphoma kinase), which is caused by specific genetic rearrangements within the tumor cells. This is not an inherited condition — the genetic changes happen only in the cancer cells during a person's lifetime. This lymphoma tends to affect younger adults, often men more than women. It usually presents as swollen lymph nodes, but it can also involve areas outside the lymph nodes such as the bones, soft tissues, spleen, or other organs. Patients may experience general symptoms like fevers, night sweats, unexplained weight loss, and fatigue — often called 'B symptoms.' The disease can behave aggressively, and many patients present at an advanced stage. Treatment typically involves combination chemotherapy, similar to regimens used for other aggressive large B-cell lymphomas. The most common first-line treatment is R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), although ALK+ LBCL cells often lack the CD20 protein that rituximab targets, so CHOP without rituximab or alternative regimens may be considered. ALK inhibitors such as crizotinib have shown promise in some cases. The prognosis is variable — some studies suggest outcomes may be somewhat worse than typical diffuse large B-cell lymphoma, while other reports indicate a more favorable course, particularly in younger patients.

Also known as:

Key symptoms:

Swollen lymph nodes that may be painlessUnexplained feversDrenching night sweatsUnexplained weight lossFatigue and low energyBone pain if bones are involvedSoft tissue masses or lumpsEnlarged spleenAbdominal pain or fullnessLoss of appetiteShortness of breath if chest lymph nodes are enlargedSkin lumps or nodules in rare cases

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

FDA & Trial Timeline

3 events
Sep 2024Emapalumab Prevention of CAR-T Cell Associated Toxicities

Marcela V. Maus, M.D.,Ph.D. — PHASE2

TrialRECRUITING
Apr 2023TTI-622 in Combination With Pembrolizumab for the Treatment of Relapsed or Refractory Diffuse Large B-Cell Lymphoma

Mayo Clinic — PHASE2

TrialACTIVE NOT RECRUITING
Apr 2023Study of SGR-1505 in Mature B-Cell Neoplasms

Schrödinger, Inc. — PHASE1

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

2 available

Kymriah

tisagenlecleucel· Novartis Pharmaceuticals Corporation■ Boxed Warning
KYMRIAH is indicated for treatment of adult patients with relapsed or refractory (r/r) large B-cell lymphoma after two or more lines of systemic therapy, including diffuse large B-cell lymphoma (DLBCL

KYMRIAH is indicated for treatment of adult patients with relapsed or refractory (r/r) large B-cell lymphoma after two or more lines of systemic therapy, including diffuse large B-cell lymphoma (DLBCL) not otherwise specified, high grade B-cell lymphoma and DLBCL arising from follicular lymphoma.

Zynlonta

loncastuximab tesirine-lpyl· ADC Therapeutics SAAccelerated Approval

indicated for the treatment of adult patients with relapsed or refractory large B-cell lymphoma after two or more lines of systemic therapy

Clinical Trials

3 recruitingView all trials with filters →
Phase 22 trials
TTI-622 in Combination With Pembrolizumab for the Treatment of Relapsed or Refractory Diffuse Large B-Cell Lymphoma
Phase 2
Active
PI: Stephen M. Ansell, MD, PhD (Mayo Clinic in Rochester) · Sites: Iowa City, Iowa; Rochester, Minnesota · Age: 1899 yrs
Emapalumab Prevention of CAR-T Cell Associated Toxicities
Phase 2
Actively Recruiting Prior treatment eligible
PI: Matthew Frigault, MD (Massachusetts General Hospital) · Sites: Boston, Massachusetts; Boston, Massachusetts · Age: 1899 yrs
Phase 11 trial
Study of SGR-1505 in Mature B-Cell Neoplasms
Phase 1
Actively Recruiting
PI: Frank G Basile, M.D. (Schrodinger Inc.) · Sites: Gilbert, Arizona; Newark, Delaware +34 more · Age: 1899 yrs

Specialists

8 foundView all specialists →
MM
Matthew Frigault, MD
Boston, Massachusetts
Specialist

Rare Disease Specialist

PI on 2 active trials
SA
Stephen M Ansell
ROCHESTER, MN
Specialist
PI on 1 active trial5 ALK-positive large B-cell lymphoma publications
FM
Frank G Basile, M.D.
RIVERSIDE, RI
Specialist
PI on 1 active trial
RL
Ryan Lynch
Specialist
PI on 3 active trials8 ALK-positive large B-cell lymphoma publications

Treatment Centers

8 centers
⚗️ Trial Site

Massachusetts General Hospital

📍 Boston, Massachusetts

👤 Matthew Frigault, MD

👤 Janssen Research & Development, LLC Clinical Trial

⚗️ Trial Site

Dana-Farber Cancer Institute

📍 Boston, Massachusetts

👤 Ann (Annie) W Silk

👤 Matthew Frigault, MD

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

Financial Resources

1 resources
Kymriah(tisagenlecleucel)Novartis Pharmaceuticals Corporation

Travel Grants

No travel grants are currently matched to ALK-positive large B-cell lymphoma.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about ALK-positive large B-cell lymphoma

Disease timeline:

New recruiting trial: Study of SGR-1505 in Mature B-Cell Neoplasms

A new clinical trial is recruiting patients for ALK-positive large B-cell lymphoma

New trial: Emapalumab Prevention of CAR-T Cell Associated Toxicities

Phase PHASE2 trial recruiting. Emapalumab

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What stage is my lymphoma, and what does that mean for my treatment plan?,Do my tumor cells express CD20, and does that affect which chemotherapy regimen is best for me?,Is an ALK inhibitor like crizotinib an option for my treatment?,Am I a candidate for stem cell transplantation if the first treatment doesn't work?,Are there any clinical trials available for ALK-positive large B-cell lymphoma?,What side effects should I expect from treatment, and how can they be managed?,What is the follow-up plan after treatment to check for relapse?

Common questions about ALK-positive large B-cell lymphoma

What is ALK-positive large B-cell lymphoma?

ALK-positive large B-cell lymphoma (also called ALK+ LBCL or ALK-positive diffuse large B-cell lymphoma) is a very rare type of non-Hodgkin lymphoma, which is a cancer of the immune system's B cells. In this disease, the cancer cells carry an abnormal activation of a protein called ALK (anaplastic lymphoma kinase), which is caused by specific genetic rearrangements within the tumor cells. This is not an inherited condition — the genetic changes happen only in the cancer cells during a person's lifetime. This lymphoma tends to affect younger adults, often men more than women. It usually presen

How is ALK-positive large B-cell lymphoma inherited?

ALK-positive large B-cell lymphoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does ALK-positive large B-cell lymphoma typically begin?

Typical onset of ALK-positive large B-cell lymphoma is adult. Age of onset can vary across affected individuals.

Are there clinical trials for ALK-positive large B-cell lymphoma?

Yes — 3 recruiting clinical trials are currently listed for ALK-positive large B-cell lymphoma on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat ALK-positive large B-cell lymphoma?

8 specialists and care centers treating ALK-positive large B-cell lymphoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.