Rare Hematology News

Disease Profile

Follicular lymphoma

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
1-5 / 10 000

33,100 - 165,500

US Estimated

1-5 / 10 000

51,350 - 256,750

Europe Estimated

Age of onset

Adult

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ICD-10

C82.0 C82.1 C82.2 C82.3 C82.4 C82.5 C82.6 C82.7 C82.9

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Lymphoma, follicular

Categories

Blood Diseases; Rare Cancers

Summary

The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
orphanet

Orpha Number: 545

Definition
Follicular lymphoma is a form of non-Hodgkin lymphoma (see this term) characterized by a proliferation of B cells whose nodular structure of follicular architecture is preserved.

Epidemiology
Prevalence of follicular lymphoma is estimated at about 1/3,000.

Clinical description
The median age at diagnosis is 60-65 years. The disease is extremely rare in children. Follicular lymphoma is located primarily in the lymph nodes, but can also involve the spleen, bone marrow, peripheral blood and Waldeyer's ring. The skin and central nervous system are affected in rare cases. Symptoms appear at an advanced stage of the disease and can include fever, night sweats and weight loss. At diagnosis, patients usually present with adenopathy and, in 50% of cases, splenomegaly.

Etiology
In 85% of cases, follicular lymphomas are associated with a translocation t(14;18) (q32;q21), which activates the BCL2 gene encoding the BCL2 protein that is essential for some apoptosis processes.

Diagnostic methods
Diagnosis is based on histological analysis of the adenopathy, a complete blood count, measurement of lactate dehydrogenase (LDH) and biopsy analysis of a lymph node. Examination using imagery (particularly a chest radiograph) is required. The observation of the translocation t(14;18) by polymerase chain reaction (PCR), in addition to suggested histological results, confirms the diagnosis.

Differential diagnosis
Differential diagnoses include chronic lymphocytic B cell leukemia, diffuse large B cell lymphoma, mantle cell lymphoma and MALT lymphoma (see these terms).

Management and treatment
If the lymphoma is localized it should be treated by radiotherapy. In the case of advanced stage lymphoma, chemotherapy with immuno-chemotherapy should be offered (rituximab combined with CHOP). In cases of chemosensitive relapse, treatment can be intensified.

Prognosis
The survival rate at five years is 80-90% and medial survival is approximately 10-12 years.

Visit the Orphanet disease page for more resources.

Symptoms

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Fever
0001945
Lymphoma
Cancer of lymphatic system
0002665
Mediastinal lymphadenopathy
Swollen lymph nodes in center of chest
0100721
Night sweats
0030166
Weight loss
0001824
30%-79% of people have these symptoms
Fatigue
Tired
Tiredness

[ more ]

0012378
Splenomegaly
Increased spleen size
0001744
5%-29% of people have these symptoms
Abnormality of the peritoneum
0002585
Lymphedema
Swelling caused by excess lymph fluid under skin
0001004
Meningitis
0001287
Pleural effusion
Fluid around lungs
0002202
Skin nodule
0200036
Percent of people who have these symptoms is not available through HPO
Bcell lymphoma
0012191
Somatic mutation
0001428

Treatment

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

  • Copanlisib(Brand name: Aliqopa) Manufactured by Bayer US LLC
    FDA-approved indication: For the treatment of adult patients with relapsed follicular lymphoma who have received at least two prior systemic therapies.
    National Library of Medicine Drug Information Portal
  • PI3K-delta and PI3K-gamma inhibitor(Brand name: Duvelisib) Manufactured by Verastem, Inc.
    FDA-approved indication: September 2018, PI3K-delta and PI3K-gamma inhibitor (Duvelisib) was approved for the treatment of adult patients with relapsed or refractory chronic lymphocytic leukemia (CLL), small lymphocytic lymphoma (SLL), or follicular lymphoma after at least two prior therapies.
    National Library of Medicine Drug Information Portal
  • Obinutuzumab(Brand name: Gazyva) Manufactured by Genentech, Inc.
    FDA-approved indication: Indicated for the treatment of patients with follicular lymphoma (FL) who relapsed after, or are refractory to, a rituximab containing regimen. Also treatment of patients achieving at least a partial remission as well as the treatment of adult patients with previously untreated stage II bulky, II or IV follicular lymphoma.
    National Library of Medicine Drug Information Portal
    Medline Plus Health Information
  • Rituximab and hyaluronidase human(Brand name: Rituxan Hycela) Manufactured by Genentech, Inc.
    FDA-approved indication: Treatment of adult patients with relapsed or refractory, follicular lymphoma as a single agent, previously untreated follicular lymphoma in combination with first line chemotherapy and, in patients achieving a complete or partial response to rituximab in combination with chemotherapy, as single-agent maintenance therapy, non-progressing (including stable disease), follicular lymphoma as a single agent after first-line cyclophosphamide, vincristine, and prednisone (CVP) chemotherapy.
    National Library of Medicine Drug Information Portal

Learn more

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Follicular lymphoma. Click on the link to view a sample search on this topic.