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
