Flashcards in Chapter 15: The Chronic lymphoid leukemias Deck (33):
What are chronic leukemias?
Diseases characterized by the accumulation of mature lymphocytes in the blood. (There is considerable overlap with lymphomas.)
How are CLLs diagnosed?
CLLs are diagnosed by chronic persistent lymphocytosis. Subtypes are diagnosed by cytogenetic, immunophenotypic, and genetic testing.
What is the most common chronic lymphoid leukemia?
Chronic lymphocytic leukemia (CML).
When is the peak incidence of CLL?
Between 60 and 80 years of age.
Where is CLL common or rare?
CLL is common in the western world but rare in the far east.
What factors predispose to CLL?
Close relatives with CLL are the biggest risk factor. Chemotherapy and radiotherapy do not seem to increase the risk as they do in other cancers.
What are the cellular characteristics of CLL?
accumualtion of relatively mature B cells with little Ig on their surface.
What is the epidemiology of CLL?
(1) 85% of cases over the age of 50
(2) Male:female ratio is 2:1
What are the clinical features of CLL?
(1) symmetrical enlargement of lymph nodes.
(4) Splenomegaly (rarely hepatomegaly)
What are the cellular findings of CLL?
(2) CD19+, CD5+, CD23+ B cells
(3) Lymphocytes are over 25% of the BM
(4) Autoimmunity against blood cells
What four chromosome abnormalities are most common with CLL?
(1) Del 13q14
(2) tirsomy 12
(3) del 11q23
(4) Structural abnormalities of 17p involving p53.
What is the prognosis for Ig genes that are unmutated (no somatic hypermutation)?
The prognosis is unfavorable for unmutated Ig genes in CLL.
What is the effect of ZAP-70 mutations on the clinical outcome and prognosis for CLL?
The prognosis is poor for CLL with ZAP-70 deficiency.
How is CLL treated?
There are no cures for CLL. Treatment is just management of symptoms including organomegaly, hemolytic episodes, anemia, and bone marrow suppression.
What drugs may be used to manage CLL?
Not all used together although fludarabine, cyclophosphamide, and rituximab may be given.
When is radiotherapy given to treat CLL?
Radiotherapy is given for CLL in late stage disease that is resistant to chemotherapy.
When is splenectomy undertaken in CLL?
When there is immune mediated cytopenia or painful enlargement of the spleen.
What differentiates Prolymphocytic leukemia from chronic lymphocytic leukemia?
(1) Promyelocytes are larger and have a large central nucleus.
(2) PLL exhibits splenomegaly without lymphadenopathy
(3) Anemia is not very common in PLL.
How is PLL treated?
Splenectomy with purine analogs and or rituximab are beneficial for PLL.
What is hairy cell leukemia?
An uncommon B cell disorder characterized by pancytopenia.
What are the presenting feature of hairy cell leukemia?
How is HCL diagnosed?
HCL is diagnosed as
(4) mild fibrosis and cellular inflitrate on bone marrow.
How is HCL treated?
(1) 2-chlorodeoxyadenosine or deoxycoformycin (90% success rate)
What is splenic marginal zone lymphoma?
It is a benign disease of the elderly characterized by massive splenomegaly and circulating monoclonal b cells with a villous appearance.
What is plasma cell leukemia?
A rare disease characterized by high numbers of circulating plasma cells. features include pancytopenia and splenomagaly
What are lymphoma/leukemia syndromes?
Lymphomas that have spread into the blood stream. Commonly non-Hodgkin lymphoma.
What is large granulocyte lymphocytic leukemia?
Leukemia associated with circulating lymphocytes that have abundant cytoplasm and azurophilic granules. (mean age of onset is 50)
What are clinical features of LGLL?
Cytopenia, neutropenia, splenomegaly, and arthropathy associated with rheuatoid arthritis.
What is adult T cell leukemia caused by?
HTLV-1 (endemic in Japan and the caribbean)
What cellular morphological characteristics are seen with ATLL?
Lymphocytes with bizarre clover leaf nuclei and CD4+ phenotype.
What is the clinical presentation of ATLL?
(1) acute onset
(3) hepatosplenomegaly and lymphadenopathy
(4) skin lesions.
What is Sezary syndrome?
Patients with Sezary syndrome will have pruritic exfoliative erythroderma on the palms of the hands and the soles of the feet. This is caused by lymphocytic inflitrate.