Initial work-up for patient suspected of hairy cell leukemia
| Recommendations . | Specific procedures and considerations . |
|---|---|
| Diagnosis and initial evaluation | |
| Complete blood count | |
| Peripheral blood smear review | Use Wright’s stain, perform white blood cell differential, and identify leukemic cells. |
| Immunophenotypic analysis by flow cytometry | Check for positivity for CD19, CD20, CD11c, CD25, CD103, CD123, CD200, and immunoglobulin light chain restriction of the circulating mononuclear cells for confirmation of diagnosis. |
| Bone marrow aspiration and trephine biopsy | Use hematoxylin and eosin stain, reticulin stain, and immunohistochemistry for CD20, and annexin-1, DBA.44, and VE1 (for BRAFV600E); identify BRAFV600E mutation by allele-specific polymerase chain reaction, sequence analysis, or immunohistochemical stain to confirm diagnosis and extent of bone marrow involvement. |
| Complete history and physical examination | Include assessment of renal function for patients in whom nucleoside analog therapy is planned. |
| Optional imaging studies | Perform chest x-ray to assess for infection; use computed tomography or ultrasound scan of abdomen to evaluate organomegaly, and lymphadenopathy. These procedures should be considered for patients in a clinical trial or those with associated symptoms referable to these systems. |
| Serology for hepatitis if planning on using an anti-CD20 monoclonal antibody | |
| Differential diagnosis | Consider hairy cell leukemia; hairy cell leukemia variant; splenic marginal zone lymphoma; splenic diffuse red pulp small B-cell lymphoma (outlines for specific immunophenotypic profiles of differential entities33-36 ). |
| Indications for treatment | |
| Hematologic parameters consistent with initiating treatment | Include at least one of the following: hemoglobin <11 g/dL, platelet count <100 000/μL, or absolute neutrophil count <1000/μL. |
| Clinical features or symptoms for which therapy may be considered | Include symptomatic organomegaly, progressive lymphocytosis, or lymphadenopathy, unexplained weight loss (>10% within prior 6 months, excessive fatigue (National Cancer Institute Common Terminology Criteria for Adverse Events grade >2). |
| Recommendations . | Specific procedures and considerations . |
|---|---|
| Diagnosis and initial evaluation | |
| Complete blood count | |
| Peripheral blood smear review | Use Wright’s stain, perform white blood cell differential, and identify leukemic cells. |
| Immunophenotypic analysis by flow cytometry | Check for positivity for CD19, CD20, CD11c, CD25, CD103, CD123, CD200, and immunoglobulin light chain restriction of the circulating mononuclear cells for confirmation of diagnosis. |
| Bone marrow aspiration and trephine biopsy | Use hematoxylin and eosin stain, reticulin stain, and immunohistochemistry for CD20, and annexin-1, DBA.44, and VE1 (for BRAFV600E); identify BRAFV600E mutation by allele-specific polymerase chain reaction, sequence analysis, or immunohistochemical stain to confirm diagnosis and extent of bone marrow involvement. |
| Complete history and physical examination | Include assessment of renal function for patients in whom nucleoside analog therapy is planned. |
| Optional imaging studies | Perform chest x-ray to assess for infection; use computed tomography or ultrasound scan of abdomen to evaluate organomegaly, and lymphadenopathy. These procedures should be considered for patients in a clinical trial or those with associated symptoms referable to these systems. |
| Serology for hepatitis if planning on using an anti-CD20 monoclonal antibody | |
| Differential diagnosis | Consider hairy cell leukemia; hairy cell leukemia variant; splenic marginal zone lymphoma; splenic diffuse red pulp small B-cell lymphoma (outlines for specific immunophenotypic profiles of differential entities33-36 ). |
| Indications for treatment | |
| Hematologic parameters consistent with initiating treatment | Include at least one of the following: hemoglobin <11 g/dL, platelet count <100 000/μL, or absolute neutrophil count <1000/μL. |
| Clinical features or symptoms for which therapy may be considered | Include symptomatic organomegaly, progressive lymphocytosis, or lymphadenopathy, unexplained weight loss (>10% within prior 6 months, excessive fatigue (National Cancer Institute Common Terminology Criteria for Adverse Events grade >2). |