HAIRY CELL LEUKEMIA

Question 1

HAIRY CELL LEUKEMIA

Answer 1

A rare neoplasm (2%)

Question 2

Ewald (1923) described it as

Answer 2

LEUKEMIC RETICULOENDOTHELIOSIS

Question 3

Characteristics:

Answer 3

Large spleen

Presence in the peripheral blood of mononuclear cells with numerous cytoplasmic processes

Question 4

Other names:

Answer 4

Reticulosis
Aleukemic reticuloendotheliosis
Reticulum cell leukemia

Question 5

PATHOPHYSIOLOGY

Mean survival =

RELATED TO CYTOPENIAS AND SPLENOMEGALY

PARANEOPLASTIC COMPLICATIONS

Answer 5

Indolent course

5 years

Two groups of patients according to complications:

Anemia
Infections
Bleeding

Autoimmune syndromes
Paraproteinemia

Question 6

CLINICAL PRESENTATION

Answer 6

Men (4-5:1)

Median age - 55 years

Splenomegaly (consistent)

Lymphadenopathy and hepatomegaly are more rarer compared to the other CLL

Question 7

LABORATORY FINDINGS

Answer 7

(+) hairy cells (

Question 8

most consistent finding

Answer 8

Pancytopenia

Question 9

most common cause of leukopenia

Answer 9

Granulocytopenia and monocytopenia

Question 10

BM

Answer 10

dry tap (due to associated fibrosis)

Question 11

BM DRY TAP

Answer 11

Lymphoid infiltrates

Increased reticulin fibers

(+) tartrate-resistant acid phosphatase
(-) for other lymphocyte markers

Question 12

CHARACTERISTICS OF A HAIRY CELL

Answer 12

Scant to abundant, agranular, light grayish blue cytoplasm

Hair like or ruffled cytoplasmic projections

Round to oval nucleus – bilobed or folded

Loose and lazy chromatin

One or more nucleoli