Mature Lymphoid Leukemia Flashcards
About what portion of chronic lymphoid leukemias are chronic lymphocytic leukemia?
90%
In what condition can “soccer ball” chromatin been seen?
Chronic Lymphocytic Leukemia
What is the immunophenotypic hallmark of CLL?
B cells expressing CD5
What is the immunophenotype of CLL?
CD19, Cd5, CD23, CD20(weak), CD11c (weak), surface light chains (weak)
True or False: Generally, CLL is a curable disease since the cells don’t divide rapidly.
False: THe low replication due to their maturity makes it hard to cure
What are the two categories of CLL based on somatic hypermutation? Which has a better prognosis
Unmutated IGHV genes (worse prognosis); Mutated IGHV genes (better prognosis)
What are the independent prognostic markers from IGHV mutations?
CD38 and ZAP70
What kind of prognosis is associated with CD38 and ZAP70 mutations? Why?
Poor prognosis bcause expression of those proteins increases BCR- mediated signaling and cell proliferation (anti-apoptosis)
What are the clinical features of Hairy Cell Leukemia?
Hepatosplenomegaly, fatigue, weakness, and recurrant infections
What lab finding is unique to hairy cell leukemia?
Peripheral monocytopenia
The presence of Tartrate-resistance acid phosphatase (TRAP) is characteristic of what condition?
Hairy cell leukemia
What is the potential effect of hairy cell leukemia on the spleen?
Hairy cells concentrate in the red pulp and cause red pulp disease causing loss of distinction between red pulp and white pulp
What is the characteristic histologic feature of red pulp disease?
Red cell “lakes”
What is the cell morphology of Hairy Leukemic Cells?
Halo or fried egg appearance
What is the characteristic immunophenotype of hairy cell leukemia?
CD11c+, CD103+, CD25+
What is the ultimate conequence of bRAF mutations in Hairy Cell Leukemia?
Activation of MAPK pathway leading to transcriptional activation of cyclin D1
What specific chemotherapy is targeted against mutant b-Raf?
Vemurafenib
What is mycosis fungoides
Epiderotropic, primary cutaneous T cell lymphoma (CTCL)
What is the morphology of mycosis fungoides histology?
IN the early phase there are band-like (lichenoid) lymphocytic infiltrate in superficial epidermis, with plaques there is increased intraepidermal collections of lymphocytes
What is the most important prognostic factor for mycosis fungoides?
Extent of cutaneous/ extracutaneous disease
What is Sezary Syndrome?
Mycosis fungoides’ cells but in the peripheral blood making the condition a leukemia
What are Sezary cells?
CLonally related neoplastic T cells with cerebriform nuclei
What is the triad of clinical features associated with sezary syndrome?
Erythroderma, generalized lymphadenopathy, presence of sezary cells in skin, lymph nodes, and peripheral blood
True or False: Generally, Sezary Syndrome is an indolent neoplasm with long life expectancies?
False- aggressive disease, overall 5 yr survival 10-20 %
