CLL and Other Chronic Disorders

Question 0

Involving T cells

Answer 0

Sézary syndrome

Question 1

Involving B cells

Answer 1

Prolymphocytic leukemia, Hairy Cell leukemia

Question 2

CLL

Findings, Development, Lymphoma Phase

Answer 2

B cell malignancy, shows bone marrow hypercellularity, absolute lymphocytosis in peripheral blood consisting of nonfunctional, homogeneous, small, hyperclumped lymphocytes and smudge cells.
Can go through “Richters Transformation” and develop into a high-grade leukemia/lymphoma
SLL – small lymphocyte lymphoma is the lymphoma phase of CLL

Question 3

Hairy cell leukemia

Frequency, Findings

Answer 3

More common in males, 7:1

B cell malignancy showing splenomegaly, bone marrow dry tap, and pancytopenia.

Question 4

Prolymphocytic leukemia

Cells involved, Findings

Answer 4

Can involve B or T cells, has marked splenomegaly due to accumulation of abnormal lymphs, shows lymphocytosis consisting mainly of prolymphocytes (along with immature granulocytes nRBCs on peripheral smear) as well as anemia and thrombocytopenia

Question 5

Multiple myeloma

Pathophysiology, Findings

Answer 5

B cell production of excessive IgG or IgA and slowed production of other immunoglobulins, causes skeletal system tumors caused by plasma cells lysing the bone, and can form aggregates/sheets of plasma cells.
Excess immunoglobulins bind platelets, blocking their function and causing prolonged bleeding.
Shows characteristic and spike in gammaglobulin region on serum electrophoresis.
The bone marrow shows >30% plasma cells
Bence-Jones proteins can be found in the urine and cause kidney damage

Question 6

Waldenström macroglobinemia

Findings

Answer 6

B cells produce excessive IgM immunoglobulins, showing plasmacytoid morphology or small cleaved cell lymphoma in bone marrow
Causes lymphadenopathy and hepatosplenomegaly
Also shows and spike in gammaglobulin region on electrophoresis as well as interfering with platelet function

Question 7

Lymphoma

Answer 7

Malignant cells form solid lymphatic tissue, starting locally but can spread initially showing lymphadenopathy, diagnosed with tissue biopsy.

Question 8

Hodgkin lymphoma

Age, Findings, Types

Answer 8

Seen in patients between 15–35 years of age or over 55, some subtypes are associated with Epstein-Barr virus.
Reed – Sternberg cells (B cell lineage) found in lymph node biopsy, usually surrounded by other white blood cells– Causing lymph node enlargement
Can be either nodular (most common) or “mixed cellularity” (highest association with Epstein-Barr virus)
Have an increased LAP during active disease

Question 9

Non-Hodgkin lymphoma

Types, Findings

Answer 9

More commonly B cell neoplasms, including Burkitt’s lymphoma phase, Mantle cell, follicular and other lymphomas
Cells range from large and young to small and mature

Question 10

Mycosis fungoides, cutaneous T-cell lymphoma

Symptoms, Assoc. Syndrome

Answer 10

Also known as T/NK cell neoplasm, causes skin itching leading to ulcerative tumors
Sézary Syndrome: a variant of mycosis fungoides that presents as a disseminated disease with widespread skin involvement and circulating lymphoma cells

Question 11

Mantle cell lymphoma

Findings

Answer 11

Cells have irregular nuclear contours, condensed chromatin, scant to moderate cytoplasm; high frequency of marrow involvement in leukemic phase; nuclei can resemble CLL or be somewhat cleaved