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Hairy Cell leukemia

Updated : April 22, 2024





Background

HCL (hairy cell leukemia) is an uncommon long-term B-cell cancer that affects the bone tissue, liver, and blood plasma. Pancytopenia, comprising monocytopenia, can be detected by a full blood count. The average age at the time of discovery is around fifty-five.

While the evidence is inconsistent, bad prognostic characteristics are age, platelets below 100, hemoglobin below 10 g/dL, absolute neutrophil count less than 1000, lymphadenopathy, and significant splenomegaly.

Other B-cell lymphoproliferative diseases, such as splenic marginal zone carcinoma, are included in the diagnostic process. Hairy cell leukemia variation (HCL-V), which is genetically distinct from Hairy cell leukemia, is also an independent component. In this disease, typical Hairy cell leukemia has a poor response.

Immunophenotypic abnormalities, the absence of a BRAF mutation, and the absence of monocytopenia can all be used to detect the variation. HCL represents about two percent of the total all leukemia cases, with about a thousand new cases reported annually in the United States.

Epidemiology

Hairy cell leukemia is uncommon cancer that accounts for about two percent of lymphoid cancer. At the time of diagnosis, the median age was 55. It is almost never noticed in youngsters, despite the fact that this would develop in young adults. With a 4:1 ratio, males are preferentially harmed compared to females.

Anatomy

Pathophysiology

Hairy cell leukemia is a clonal lymphoproliferative neoplasm. The presence of the V600E BRAF alteration in the great majority of patients with traditional HCL has led to leukemia on that the RAS-RAF-MAPK signaling pathway is signaling in the disease’s etiology.

Enhanced cell multiplication and viability, as well as malignancy, are the results of this pathway’s constant action. CDKN1B inactivation was also discovered in 16 percent of patients with classical HCL in a new analysis by Sascha Dietrich et al. In Hairy cell leukemia, it is the second most frequently altered chromosome.

Etiology

Hairy cell leukemia has an etiology that is unknown. Prior exposure to certain substances, on the other hand, may play a role in its development. Most cases are thought to be caused by a mutation in the V600 BRAF gene in late activated memory B cells.

Genetics

Prognostic Factors

Without therapy, the median survival time is usually 4 years. Cases with HCL with VH4-34 positivity have a bad prognosis.

While the evidence is inconsistent, poor prognostic factors include age, hemoglobin less than 10g/dl, platelets less than 100, ANC less than 1000, lymphadenopathy, and significant splenomegaly.

Other B-cell lymphoproliferative diseases, such as splenic marginal zone lymphoma, are included in the differential diagnosis.

Clinical History

Physical Examination

Age group

Associated comorbidity

Associated activity

Acuity of presentation

Differential Diagnoses

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

Medication

 

cladribine

0.09 - 0.1

mg/kg

Intravenous (IV)

continuous infusion

7

days

or 0.15 mg/kg/day IV over 2 hr for 5 days in combination with rituximab



 
 

Media Gallary

References

https://www.ncbi.nlm.nih.gov/books/NBK499845/
Page Break

Hairy Cell leukemia

Updated : April 22, 2024




HCL (hairy cell leukemia) is an uncommon long-term B-cell cancer that affects the bone tissue, liver, and blood plasma. Pancytopenia, comprising monocytopenia, can be detected by a full blood count. The average age at the time of discovery is around fifty-five.

While the evidence is inconsistent, bad prognostic characteristics are age, platelets below 100, hemoglobin below 10 g/dL, absolute neutrophil count less than 1000, lymphadenopathy, and significant splenomegaly.

Other B-cell lymphoproliferative diseases, such as splenic marginal zone carcinoma, are included in the diagnostic process. Hairy cell leukemia variation (HCL-V), which is genetically distinct from Hairy cell leukemia, is also an independent component. In this disease, typical Hairy cell leukemia has a poor response.

Immunophenotypic abnormalities, the absence of a BRAF mutation, and the absence of monocytopenia can all be used to detect the variation. HCL represents about two percent of the total all leukemia cases, with about a thousand new cases reported annually in the United States.

Hairy cell leukemia is uncommon cancer that accounts for about two percent of lymphoid cancer. At the time of diagnosis, the median age was 55. It is almost never noticed in youngsters, despite the fact that this would develop in young adults. With a 4:1 ratio, males are preferentially harmed compared to females.

Hairy cell leukemia is a clonal lymphoproliferative neoplasm. The presence of the V600E BRAF alteration in the great majority of patients with traditional HCL has led to leukemia on that the RAS-RAF-MAPK signaling pathway is signaling in the disease’s etiology.

Enhanced cell multiplication and viability, as well as malignancy, are the results of this pathway’s constant action. CDKN1B inactivation was also discovered in 16 percent of patients with classical HCL in a new analysis by Sascha Dietrich et al. In Hairy cell leukemia, it is the second most frequently altered chromosome.

Hairy cell leukemia has an etiology that is unknown. Prior exposure to certain substances, on the other hand, may play a role in its development. Most cases are thought to be caused by a mutation in the V600 BRAF gene in late activated memory B cells.

Without therapy, the median survival time is usually 4 years. Cases with HCL with VH4-34 positivity have a bad prognosis.

While the evidence is inconsistent, poor prognostic factors include age, hemoglobin less than 10g/dl, platelets less than 100, ANC less than 1000, lymphadenopathy, and significant splenomegaly.

Other B-cell lymphoproliferative diseases, such as splenic marginal zone lymphoma, are included in the differential diagnosis.

cladribine

0.09 - 0.1

mg/kg

Intravenous (IV)

continuous infusion

7

days

or 0.15 mg/kg/day IV over 2 hr for 5 days in combination with rituximab



https://www.ncbi.nlm.nih.gov/books/NBK499845/
Page Break