Chapter 15: The Chronic lymphoid leukemias

Question 1

What are chronic leukemias?

Answer 1

Diseases characterized by the accumulation of mature lymphocytes in the blood. (There is considerable overlap with lymphomas.)

Question 2

How are CLLs diagnosed?

Answer 2

CLLs are diagnosed by chronic persistent lymphocytosis. Subtypes are diagnosed by cytogenetic, immunophenotypic, and genetic testing.

Question 3

What is the most common chronic lymphoid leukemia?

Answer 3

Chronic lymphocytic leukemia (CML).

Question 4

When is the peak incidence of CLL?

Answer 4

Between 60 and 80 years of age.

Question 5

Where is CLL common or rare?

Answer 5

CLL is common in the western world but rare in the far east.

Question 6

What factors predispose to CLL?

Answer 6

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.

Question 7

What are the cellular characteristics of CLL?

Answer 7

accumualtion of relatively mature B cells with little Ig on their surface.

Question 8

What is the epidemiology of CLL?

Answer 8

(1) 85% of cases over the age of 50

(2) Male:female ratio is 2:1

Question 9

What are the clinical features of CLL?

Answer 9

(1) symmetrical enlargement of lymph nodes.
(2) Anemia
(3) thrombocytopenia
(4) Splenomegaly (rarely hepatomegaly)
(5) Immunosuppression

Question 10

What are the cellular findings of CLL?

Answer 10

(1) lymphocytosis
(2) CD19+, CD5+, CD23+ B cells
(3) Lymphocytes are over 25% of the BM
(4) Autoimmunity against blood cells

Question 11

What four chromosome abnormalities are most common with CLL?

Answer 11

(1) Del 13q14
(2) tirsomy 12
(3) del 11q23
(4) Structural abnormalities of 17p involving p53.

Question 12

What is the prognosis for Ig genes that are unmutated (no somatic hypermutation)?

Answer 12

The prognosis is unfavorable for unmutated Ig genes in CLL.

Question 13

What is the effect of ZAP-70 mutations on the clinical outcome and prognosis for CLL?

Answer 13

The prognosis is poor for CLL with ZAP-70 deficiency.

Question 14

How is CLL treated?

Answer 14

There are no cures for CLL. Treatment is just management of symptoms including organomegaly, hemolytic episodes, anemia, and bone marrow suppression.

Question 15

What drugs may be used to manage CLL?

Answer 15

(1) Chlorambucil
(2) Fludarabine
(3) Alemtuzumab
(4) Rituximab
(5) Corticosteroids
Not all used together although fludarabine, cyclophosphamide, and rituximab may be given.

Question 16

When is radiotherapy given to treat CLL?

Answer 16

Radiotherapy is given for CLL in late stage disease that is resistant to chemotherapy.

Question 17

When is splenectomy undertaken in CLL?

Answer 17

When there is immune mediated cytopenia or painful enlargement of the spleen.

Question 18

What differentiates Prolymphocytic leukemia from chronic lymphocytic leukemia?

Answer 18

(1) Promyelocytes are larger and have a large central nucleus.
(2) PLL exhibits splenomegaly without lymphadenopathy
(3) Anemia is not very common in PLL.

Question 19

How is PLL treated?

Answer 19

Splenectomy with purine analogs and or rituximab are beneficial for PLL.

Question 20

What is hairy cell leukemia?

Answer 20

An uncommon B cell disorder characterized by pancytopenia.

Question 21

What are the presenting feature of hairy cell leukemia?

Answer 21

(1) male
(2) infections
(3) anemia
(4) splenomegaly
(5) pancytopenia.

Question 22

How is HCL diagnosed?

Answer 22

HCL is diagnosed as

(1) CD22+
(2) CD103+
(3) FMC7+
(4) mild fibrosis and cellular inflitrate on bone marrow.

Question 23

How is HCL treated?

Answer 23

(1) 2-chlorodeoxyadenosine or deoxycoformycin (90% success rate)
(2) Alpha-interferon

Question 24

What is splenic marginal zone lymphoma?

Answer 24

It is a benign disease of the elderly characterized by massive splenomegaly and circulating monoclonal b cells with a villous appearance.

Question 25

What is plasma cell leukemia?

Answer 25

A rare disease characterized by high numbers of circulating plasma cells. features include pancytopenia and splenomagaly

Question 26

What are lymphoma/leukemia syndromes?

Answer 26

Lymphomas that have spread into the blood stream. Commonly non-Hodgkin lymphoma.

Question 27

What is large granulocyte lymphocytic leukemia?

Answer 27

Leukemia associated with circulating lymphocytes that have abundant cytoplasm and azurophilic granules. (mean age of onset is 50)

Question 28

What are clinical features of LGLL?

Answer 28

Cytopenia, neutropenia, splenomegaly, and arthropathy associated with rheuatoid arthritis.

Question 29

What is adult T cell leukemia caused by?

Answer 29

HTLV-1 (endemic in Japan and the caribbean)

Question 30

What cellular morphological characteristics are seen with ATLL?

Answer 30

Lymphocytes with bizarre clover leaf nuclei and CD4+ phenotype.

Question 31

What is the clinical presentation of ATLL?

Answer 31

(1) acute onset
(2) hypercalcemia
(3) hepatosplenomegaly and lymphadenopathy
(4) skin lesions.

Question 32

What is Sezary syndrome?

Answer 32

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.

Question 33

What are sezary cells?

Answer 33

Lymphocytes with deep nuclear clefting similar to ATLL.