HCL Flashcards
Typical immunophenotype for cHCL:
CD5-, CD10-, CD11c+, CD20+ (bright), CD22+, CD25+, CD103+, CD123+, cyclin D1+, annexin A1+, and CD200+ (bright)
Immunophenotype for HCLv (ICC)/SBLPN (WHO5):
CD25-, CD123-, annexin A1-, and negative for BRAF V600E mutations
HCLv (ICC)/SBLPN (WHO5) and IGHV4-34–mutant HCL often show mutations in
MAP2K1
IGHV4-34 rearrangements: poor prognosis
Indications for treatment:
- Systemic symptoms
- Unexplained weight loss (>10% within prior 6 months)
- Excessive fatigue
- Recurrent infection
- Hemoglobin <11 g/dL
- Platelets <100,000/mcL
- Absolute neutrophil count (ANC) <1000/mcL
- Symptomatic organomegaly
- Progressive lymphocytosis or lymphadenopathy
Preferred Regimens for Initial therapy
Purine analogs
* Cladribine ± rituximab
* Pentostatin
Considered for patients who are unable to tolerate purine analogs including frail patients and those with active infection
Vemurafenib ± anti-CD20 monoclonal antibody (mAb)
BRAF inhibitor
- Vemurafenib
- Dabrafenib
Preferred Regimens for R/R Less than complete
response after initial treatment OR Relapse < 2 years
- Clinical trial
- Dabrafenib + trametinib (if not previously treated with BRAF inhibitor)
- Vemurafenib ± rituximab (if not previously given)
Preferred Regimens for R/R ≥2 years
- Retreatment with initial purine analog + rituximab
- Alternative purine analog + rituximab
Considered for patients with disease resistant to BRAF inhibitor therapy
Venetoclax ± rituximab
TRUE OR FALSE
Standard-dose purine analogs should not be administered to patients with active life-threatening or chronic infection.
TRUE
Standard-dose purine analogs should not be administered to patients with active life-threatening or chronic infection.
Treat active infection prior to initiating treatment with standard-dose purine analogs.
If it is not possible to control infection, consider initiating treatment with low-dose pentostatin before using standard-dose purine analogs to secure a durable response.
HCL RESPONSE CRITERIA
Complete response (CR)
Near normalization of peripheral blood counts:
* Hemoglobin >11 g/dL (without transfusion)
* Platelets >100,000/mcL
* ANC >1500/mcL.
* Regression of splenomegaly on physical examination.
* Absence of morphologic evidence of HCL on both the peripheral blood smear and the bone marrow examination.
HCL RESPONSE CRITERIA
Partial response (PR)
Near normalization of the peripheral blood count (as in CR) with a minimum of 50% improvement in
organomegaly and bone marrow biopsy infiltration with HCL.
HCL RESPONSE CRITERIA
Stable disease (SD)
Patients whose disease has not met the criteria for an objective remission after therapy are considered to have SD.
SD is not an acceptable response.
HCL RESPONSE CRITERIA
Progressive disease (PD)
Patients who have an increase in symptoms related to disease, a 25% increase in organomegaly, or a 25% decline in their hematologic parameters
HCL RESPONSE CRITERIA
Relapse
Reappearance of HCL in the peripheral blood, the bone marrow biopsy, or both by morphologic stains in the absence of hematologic relapse
Hematologic relapse is defined as reappearance of cytopenia(s) below the thresholds defined above for CR and PR.
No treatment is necessarily needed in case of morphologic relapse, treatment decisions for a hematologic relapse are based on several parameters (eg, hematologic parameters warranting intervention, reoccurrence of disease-related symptoms).
In patients treated with cladribine, evaluation should not be done before _____ months after therapy.
4 months
In patients being treated with pentostatin, the bone marrow can be evaluated
After the blood counts have nearly normalized and the physical examination shows no splenomegaly
Duraion of herpes and pneumocystis jirovecii pneumonia (PJP) virus prophylaxis
Minimum of 3 months and until CD4+ T-cell counts ≥200 cells/μL
Hairy cell leukemia (HCL) is a rare type of indolent B-cell leukemia comprising about ______ % of all lymphoid leukemias.
2%
Symptoms of HCL
Fatigue and weakness, and most patients will present with splenomegaly (symptomatic or asymptomatic) and/or hepatomegaly, pancytopenia, and uncommonly peripheral lymphadenopathy
Morphology of HCL
- Small to medium in size, and show a round, oval, or indented nucleus with a well-defined nuclear border.
- Cytoplasm with prominent hair-like projections of the cytoplasmic membrane
- Increased reticulin fibrosis, which frequently results in a “dry” tap
New name of HCLv
Splenic Bcell lymphoma/leukemia with prominent nucleoli (SBLPN)
More aggressive disease course and may not respond to standard HCL therapies
TRUE OR FALSE
Unmutated IGHV may serve as a prognostic marker for poorer outcomes with conventional
therapies
TRUE
Unmutated IGHV may serve as a prognostic marker for poorer outcomes with conventional
therapies
Since it is associated with primary refractoriness to purine analog monotherapy, more rapid disease progression, and poor survival
Mutation identified in the majority of patients with cHCL and is now regarded as the main source of pathogenesis
BRAF V600E kinase-activating mutation
HCLv (ICC)/SBLPN (WHO5) and B-cell lymphoproliferative neoplasms with a cHCL phenotype expressing IGHV4-34 rearrangement lack BRAF V600E mutation
TRUE OR FALSE
At the present time, there are no established treatment options for the optimal frontline or subsequent treatment of patients with HCLv
(ICC)/SBLPN (WHO5).
TRUE
At the present time, there are no established treatment options for the optimal frontline or subsequent treatment of patients with HCLv
(ICC)/SBLPN (WHO5).
TRUE OR FALSE
IV cladribine was associated with a lower rate of viral infections and mucositis despite having a higher rate of neutropenia
FALSE
Subcutaneous cladribine was associated with a lower rate of viral infections and mucositis despite having a higher rate of neutropenia
(CD22-directed recombinant immunotoxin)
was initially approved for the treatment of relapsed or refractory HCL but currently no longer
recommended
Moxetumomab pasudotox
