Hairy cell leukemia Flashcards Preview

Hematology, oncology > Hairy cell leukemia > Flashcards

Flashcards in Hairy cell leukemia Deck (26)
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1
Q

Gene mutation seen in virtually all cases of HCL

A

BRAF V600E

2
Q

Presentation of HCL

A

Systemic complaints (25%) (fatigue, weakness, weight loss)
Bruising and bleeding (25%)
*B symptoms and adenopathy are uncommon
Abdominal fullness or discomfort (splenomegaly)
Asymptomatic (25%)

3
Q

diagnosis

A

BMB + flow cytometry and Typical immunophenotype of peripheral blood (usually dry tap with BMB) + typical clinical manifestations

4
Q

what is hairy cell variant of HCL?

A

variant that tends not to respond as well to purine nucleoside analog based therapy

5
Q

Indications for initiating therapy in HCL

A

symptomatic splenomegaly, b symptoms, recurrent infections, cytopenias (hgb<12, platelet count <100, ANC<1)

6
Q

efficacy of treatment for HCL

A

CR is achieved in most (70-90%)

relapse rate of approximately 30-40%)

7
Q

Treatment options for HCL

A

1) Cladribine

2) Pentostatin

8
Q

Typical response to therapy

A

Improvement in peripheral blood counts may require weeks to months.

9
Q

general treatment strategy

A

Observe if asymptomatic or no indications for treatment
Wait until symptomatic or severely cytopenic –> induction therapy single cycle of cladribine –> consolidation therapy

10
Q

cell of origin

A

B cell neoplasm

11
Q

clinical behavior

A

indolent

12
Q

Demographic

A

Males (4:1)

caucasions (3:1)

13
Q

Lab findings

A

Pancytopenia (60-80%)

14
Q

BMB findings with hairy cell

A
  • commonly dry tap due to HCL-induced marrow fibrosis
15
Q

What is a trephine biopsy?

A
  • tiny core of bone marrow tissue is removed then sliced very thinly.
16
Q

Infection disease hairy cell leukemia patients are at increased risk for

A

Nontuberculous mycobacterial infections

17
Q

Major cause of death in patients with HCL

A

Infection

18
Q

Medication class of pentostatin and cladribine

A

Purine analogues (antimetabolites)

19
Q

SE to know about with purine analogs

A

Profound neutropenia + lymphopenia (suppress immune effector cells)

20
Q

typical induction therapy

A

Single cycle of cladribine (5 or 7 day infusion)

21
Q

Consolidation therapy

A

Short course of rituximab

22
Q

Management of symptomatic splenomegaly

A
  • splenectomy as palliative therapy

- salvage splenectomy if persistently pancytopenic

23
Q

Response assessment

A
  • Serial CBCs for count recovery

- Monitor for signs of infection

24
Q

Typical response to purine analogs

A

Durable responses are seen in >90 percent of patients with a median progression-free survival (PFS) of 9 to 11 years.

25
Q

Primary major contraindication to purine analogs

A

active infection (given myelosuppression)

26
Q

markers

A

CD11C+

CD103+

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