Overview
Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a very rare type of blood cancer that mainly affects the spleen. It is sometimes also called splenic diffuse red pulp small B-cell lymphoma, unclassifiable, and it belongs to a group of cancers known as B-cell non-Hodgkin lymphomas. In this disease, abnormal small B-cells (a type of white blood cell) build up in the red pulp of the spleen — the part of the spleen that filters blood. This causes the spleen to become enlarged, a condition called splenomegaly. Patients often also have abnormal lymphoma cells circulating in their blood and may have involvement of the bone marrow and liver. Common symptoms include an enlarged spleen that may cause discomfort or fullness in the upper left abdomen, fatigue, low blood cell counts (which can lead to anemia, easy bruising, or increased infections), and sometimes mild fevers or night sweats. Unlike some other splenic lymphomas, SDRPL typically does not cause enlarged lymph nodes elsewhere in the body. This lymphoma tends to follow a slow (indolent) course in many patients. The most common and often effective treatment is surgical removal of the spleen (splenectomy), which can lead to long-lasting remission. In some cases, chemotherapy or immunotherapy may be considered, particularly if the disease progresses after splenectomy or if surgery is not an option. Because this disease is so rare, it was only recently recognized as a distinct entity by the World Health Organization, and research is still ongoing to better understand its biology and optimal treatment.
Also known as:
Key symptoms:
Enlarged spleen (feeling of fullness or pain in the upper left belly)Fatigue and tirednessLow red blood cell count (anemia), causing weakness and pale skinLow platelet count, leading to easy bruising or bleedingLow white blood cell count, increasing risk of infectionsAbnormal lymphoma cells found in the bloodNight sweatsUnexplained weight lossMild feversBone marrow involvementLiver enlargement in some cases
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Splenic diffuse red pulp small B-cell lymphoma.
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Specialists
View all specialists →No specialists are currently listed for Splenic diffuse red pulp small B-cell lymphoma.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Splenic diffuse red pulp small B-cell lymphoma.
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Caregiver Resources
NORD Caregiver Resources
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Mental Health Support
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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.Is splenectomy the best treatment option for me, and what are the risks and benefits?,How will we monitor my disease after treatment to check for recurrence?,What vaccinations and preventive measures do I need if my spleen is removed?,Are there clinical trials available for my specific type of lymphoma?,What symptoms should prompt me to seek emergency care?,How does this lymphoma differ from other types of splenic lymphoma, and does that affect my treatment?,What is the likelihood that my disease could come back or become more aggressive over time?
Common questions about Splenic diffuse red pulp small B-cell lymphoma
What is Splenic diffuse red pulp small B-cell lymphoma?
Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a very rare type of blood cancer that mainly affects the spleen. It is sometimes also called splenic diffuse red pulp small B-cell lymphoma, unclassifiable, and it belongs to a group of cancers known as B-cell non-Hodgkin lymphomas. In this disease, abnormal small B-cells (a type of white blood cell) build up in the red pulp of the spleen — the part of the spleen that filters blood. This causes the spleen to become enlarged, a condition called splenomegaly. Patients often also have abnormal lymphoma cells circulating in their blood and
How is Splenic diffuse red pulp small B-cell lymphoma inherited?
Splenic diffuse red pulp small B-cell lymphoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Splenic diffuse red pulp small B-cell lymphoma typically begin?
Typical onset of Splenic diffuse red pulp small B-cell lymphoma is adult. Age of onset can vary across affected individuals.