Follicular lymphoma

Other Names:

Lymphoma, follicular

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. 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.

Follicular lymphoma replacing a lymph node; the bubble-like outgrowths are enlarged follicles.

Symptoms

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. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. 80%-99% of people have these symptoms

• Fever
• Lymphoma(Cancer of lymphatic system)
• Mediastinal lymphadenopathy(Swollen lymph nodes in center of chest)
• Night sweats
• Weight loss

30%-79% of people have these symptoms

• Fatigue
• Tired
• Splenomegaly(Increased spleen size)

5%-29% of people have these symptoms

• Abnormality of the peritoneum
• Lymphedema(Swelling caused by excess lymph fluid under skin)
• Meningitis
• Pleural effusion(Fluid around lungs)
• Skin nodule

Epidemiology

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

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.

Diagnosis

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 diagnoses include chronic lymphocytic B cell leukemia, diffuse large B cell lymphoma, mantle cell lymphoma and MALT lymphoma

According to World Health Organization (WHO) criteria, differences in the microscopically-determined morphology of these tissues can be used to diagnose and categorized FL into the following 3 Grades with grade 3 having A and B subtypes

• Grade 1: follicles have <5 centroblasts per high-power field (hpf).
• Grade 2: follicles have 6 to 15 centroblasts per hpf.
• Grade 3: follicles have >15 centroblasts per hpf.
  • Grade 3A: Grade 3 in which the follicles contain predominantly centrocytes.
  • Grade 3B: Grade 3 in which the follicles consist almost entirely of centroblasts.

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

• Copanlisib (Brand name: Aliqopa) For the treatment of adult patients with relapsed follicular lymphoma who have received at least two prior systemic therapies.
• PI3K-delta and PI3K-gamma inhibitor (Brand name: Duvelisib)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.
• Obinutuzumab (Brand name: Gazyva)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.
• Rituximab and hyaluronidase human (Brand name: Rituxan Hycela) 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.

Prognosis

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

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