Chronic lymphocytic leukemia

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Other Names: Leukemia, lymphocytic, chronic; Chronic B-Cell Lymphocytic Leukemia; B Cell Chronic Lymphocytic Leukemia

chronic lymphocytic leukemia (KRAH-nik LIM-foh-SIH-tik loo-KEE-mee-uh) is an indolent (slow-growing) cancer in which too many immature lymphocytes (white blood cells) are found mostly in the blood and bone marrow. Sometimes, in later stages of the disease, cancer cells are found in the lymph nodes and the disease is called small lymphocytic lymphoma. Also called CLL.

A diagram showing the cells affected by CLL

Signs and symptoms

Early signs and symptoms may include swollen lymph nodes, fatigue, weight loss, fever, night sweats and/or frequent infections.

The following list includes the most common signs and symptoms in people with chronic lymphocytic leukemia (CLL). Signs and symptoms may include:

  • Enlarged lymph nodes
  • Enlarged liver and/or spleen
  • Night sweats
  • Fever
  • Fatigue
  • Loss of appetite
  • Weight loss
  • Frequent infections
  • Abnormal bruising

Symptoms usually develop slowly, although CLL can progress quickly in rare cases. Bone marrow changes may lead to low blood oxygen (anemia) and a low platelet count (thrombocytopenia). The severity of CLL may depend on genetic and other factors, including the presence of underlying health conditions. It is thought that CLL occurs following a pre-cancerous condition known as monoclonal B-cell lymphocytosis (MBL). MBL is present in about 10 percent of healthy adults. Only a small percentage of people with MBL develop CLL.

Risk factors

CLL usually occurs in adults around the age of 70 and begins in the bone marrow and then spreads to the blood. Other risk factors include age, exposure to pesticides or herbicides, allergic conditions, and a current hepatitis C infections.

Cause

The underlying cause of chronic lymphocytic leukemia (CLL) is not known. About 10% of people with CLL have a family history of this condition.

Diagnosis

Chronic lymphocytic leukemia (CLL) is diagnosed through a clinical examination and specific blood tests. Sometimes CLL is first detected by chance during a routine blood test. Tests that may be used to confirm a diagnosis of CLL include a blood count, peripheral blood smear, flow cytometry, and a biopsy of the bone marrow or lymph nodes.

Treatment

Treatment for chronic lymphocytic leukemia (CLL) depends on many factors. These include the symptoms, the stage of the CLL, other diseases that are present, and whether the CLL has come back. People with early stage CLL who have no symptoms may simply be closely monitored by their doctor without any treatment. Treatment options may include, chemotherapy, radiation therapy, and targeted therapy (drugs used to find and kill cancer cells without hurting normal cells). In younger patients, bone marrow or stem cell transplantation may be an option. Specialists involved in the care of someone with CLL may include:

  • Medical oncologist
  • Radiation oncologist
  • Hematologist
  • Surgeon

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

  • Ofatumumab (Brand name: Arzerra)Arzerra in combination with chlorambucil, for the treatment of previously untreated patients with chronic lymphocytic leukemia (CLL) for whom fludarabine-based therapy is considered inappropriate. Also Arzerra in combination with fludarabine and cyclophosphamide for the treatment of patients with relapsed chronic lymphocytic leukemia (CLL).
  • Acalabrutinib (Brand name: CALQUENCE)CALQUENCE is indicated for the treatment of adult patients with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL).

Alemtuzumab (Brand name: Campath)The treatment of patients with B-cell chronic lymphocytic leukemia who have been treated with alkylating agents and who have failed fludarabine therapy.

  • 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.

Buffered intrathecal electrolyte/dextrose injection (Brand name: Elliotts B Solution) -For the intrathecal administration of methotrexate and cytarabine for the prevention or treatment of meningeal leukemia and lymphocytic lymphoma.

Fludarabine phosphate oral tablets (Brand name: Fludarabine (oral))Treatment of adult patients with B-cell chronic lymphocytic leukemia (CLL) whose disease has not responded to or has progressed during or after treatment with at least one standard alkylating-agent containing regimen.

  • Obinutuzumab (Brand name: Gazyva) Treatment of patients with previously untreated chronic lymphocytic leukemia in combination with chlorambucil.
  • Ibrutinib (Brand name: Imbruvica)Treatment of patients with chronic lymphocytic leukemia (CLL) who have received at least one prior therapy. Treatment of patients with chronic lymphocytic leukemia with or without 17p deletion.
  • Rituximab and hyaluronidase human (Brand name: Rituxan Hycela)Treatment of adult patients with previously untreated and previously treated CLL in combination with fludarabine.
  • Bendamustine hydrochloride (Brand name: Treanda)
  • Venetoclax (Brand name: Venclexta)enetoclax (Venclexta) received expanded approval for the treatment of patients with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL), with or without 17p deletion, who have received at least one prior therapy.
  • idelalisib (Brand name: Zydelig)

Statistics

It is estimated that about 4 in 100,000 people are diagnosed with chronic lymphocytic leukemia (CLL) every year in the US and Europe.

NIH genetic and rare disease info

Chronic lymphocytic leukemia is a rare disease.

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