Hairy Cell Leukemia, CLL, Flashcards
chronic lymphocytic leukemia
Features of Hairy cell leukemia?
pancytopenia due to bone marrow fibrosis, granulocytoepenia (resulting in infections), anemia (weakness and fatigue and thrombocytopenia See splenomegaly - early satiety hepatomegaly or LAD
diagnosis of hairy cell leukemia? on peripheral smear on bone marrow biopsy flow cytometry
peripheral smear - see positive TRAP staining, see “fried egg appearance” of lymphoid cells and bone marrow biopsy - hair like projections in bone marrow biopsy - see dry tap flow cytometry is positive for CD25, CD11c, and CD22
treatment of hairy cell leukemia
cladribine _ rituximab chemotherapy (for moderate/severe) life expectancy often near normal
what causes hairy cell leukemia?
BRAF V600E and see clonal B cell neoplasm and seen in middle age older adults.
what is seen on peripheral smear for hair cell leukemia
hairy projections on lymphocyte that has pale blue cytoplasm and larger than normal size. + TRAP staining “Fried egg appearance” of lymphoid cells
what medical condition is associated with chronic lymphocytic leukemia (CLL)
With CLL, see lymphocytes (that don’t function) that are small, mature smudge cells.
see hypogammoglobulinemia so see recurrent infections
see autoantibodies - see warm autoimmune hemolytic anemia
see autoimmune hemolytic anemia which can cause extravascular red blood cell destruction without hyperkalemia.
test for presence with a Coomb’s test (will be positive)
Tx AIHA with steroids.
flow cytometry = T cell marker CD5 + B cell markers CD20 & CD23.
when do we use fludarabine based chemotherapy?
for treatment of chronic lymphocytic leukemia.
when do we use lenalidomide for treatment?
for MM and MDS with 5q - syndrome.
Lenalidomide is a immunodulator and anti-angiogenic agent
When do we use Anti - CD20 antibodies (like rituximab?)
treatment of CD20 positive B cell lymphomas, RA, and microscopic polyangiitis.
CLL or chronic lymphocytic leukemia is
(clinical facts)
called the “asymptomatic with lymphocytosis on CBC”
median age 70 yrs
30% of all leukemias in US presents with B symptoms anemia thrombocytopenia, painless LAD, and hepatosplenomegaly
- see “smudge cells” and small mature lymphocytes
- see hypoglobulinemia - so there’s lots of infections
- see warm autoimmune hemolytic anemia - because of autoantibodies
- see easy bruising.
flow cytometry = T cell marker CD5 + B cell markers CD20 & CD23.
Diagnosis of CLL or chronic lymphocytic leukemia is
peripheral smear >5000 /mm^3 of mature appearing small B lymphocytes Flow cytometry has monoclonal B cell characteristics of CD19, CD20, and CD23 no need for bone marrow biopsy or lymph node excision.
what concurrent medical condition is seen commonly with CLL or chronic lymphocytic leukemia
see autoimmune hemolytic anemia AIHA in 11% especialy in those treated with purine analogs (fludarabine, cladribine, pentostatin) see spherocytes on peripheral smear also see ITP very often can see hypogammoglobunemia in later dx. See recurrent infections.
why do we see spherocytes with CLL or chronic lymphocytic leukemia
because there’s autoimmune hemolytic anemia and the autoantibodies are detected by phagocytes and there is partial RBC phagocytosis by the splenic macrophages causes excessive surface membrane loss see spherocytosis
Diagnosis of autoimmune hemolytic anemia
see positive DAT or direct antiglobulin test (Coomb’s) test which detects IgG or complement on the RBC surface.
Treatment of the AIHA
steroids to start remission and splenectomy if steroids are not effective
if presenting with acute onset of severe hemolysis and symptomatic anemia, need to give immediate pRBC transfusion
Hairy cell leukemia presentation What is seen on peripheral smear?
cytopenia and splenomegaly without LAD Blood smear is positive for CD25, CD11c and CD 22 with positive stains for TRAP (tartrate resistant acid phosphatase) see “fried egg appearance” of lymphocytes
if pt with CLL or chronic lymphocytic leukemia has no symptoms then
don’t treat. high value care question. CLL has an indolent phase with median survival of >10 years in asympotmatic pts. Don’t need treatment and can be observed. Tx should be started with disease progression and advanced stage CLL as they have a median survival time without CLL treatment. can get chemotherapy.
Treatment of CLL or chronic lymphocytic leukemia is by:
curative therapy: stem cell transplant but this is limited by age
combo chemotherapy with: rituximab or benamustine + rutiximab or ibrutinib
don’t treat if asymptomatic
if symptomatic treat:
treating if <65 years old: ibrutinib +/- rituximab
what is a complication of symptomatic CLL or chronic lymphocytic leukemia?
can develop infections or chronic pneumonias So screen for hypogammoglobunemia (66%) of pts in later disease.
persistent lymphocytosis with absolute lymphocyte count >5K (can be elevated to >100K) LAD Splenomegaly hepatomegaly mild neutropenia, anemia and thrombocytopenia
Think chronic lymphocytic leukemia
most common adult leukemia in western countries
chronic lymphocytic leukemia
diagnosis of chronic lymphocytic leukemia
peripheral smear and flow cytometry of peripheral blood don’t need lymph node biopsy or bone marrow aspiration or biopsy to diagnose it.
when do you get a CLL lymph node biopsy?
when there’s concern for a Richter transformation to an aggressive large cell lymphoma
CLL has what on peripheral smear
smudge cells + positive lymphadenopathy
can see infections and lots of lymphocytes
when do you start chemotherapy for CLL?
what is treatment for CLL?
start chemotherapy if you see Hgb<10 or Plts <100K
generally you observe unless they are symptomatic.
if symptomatic and <65 years old give ibrutinib +/- rituximab.
Give IVIG for infections.
Indications for treatment of CLL or chronic lymphocytic leukemia?
lymphocytosis alone -> no treatment
lymphocytosis + Hgb<11 -> treat
lymphocytosis + thrombocytopenia <100K -> treat
tx with ibrutinib +/- rituximab
