Find information on thousands of medical conditions and prescription drugs.

Hairy cell leukemia

Hairy cell leukemia is a slow growing leukemia. It is fairly rare with about 1000 cases diagnosed each year in the United States. It is most common in older white males. It is a type of Chronic lymphoid leukemia. more...

Home
Diseases
A
B
C
D
E
F
G
H
Hairy cell leukemia
Hallermann Streiff syndrome
Hallux valgus
Hantavirosis
Hantavirus pulmonary...
HARD syndrome
Harlequin type ichthyosis
Harpaxophobia
Hartnup disease
Hashimoto's thyroiditis
Hearing impairment
Hearing loss
Heart block
Heavy metal poisoning
Heliophobia
HELLP syndrome
Helminthiasis
Hemangioendothelioma
Hemangioma
Hemangiopericytoma
Hemifacial microsomia
Hemiplegia
Hemoglobinopathy
Hemoglobinuria
Hemolytic-uremic syndrome
Hemophilia A
Hemophobia
Hemorrhagic fever
Hemothorax
Hepatic encephalopathy
Hepatitis
Hepatitis A
Hepatitis B
Hepatitis C
Hepatitis D
Hepatoblastoma
Hepatocellular carcinoma
Hepatorenal syndrome
Hereditary amyloidosis
Hereditary angioedema
Hereditary ataxia
Hereditary ceroid...
Hereditary coproporphyria
Hereditary elliptocytosis
Hereditary fructose...
Hereditary hemochromatosis
Hereditary hemorrhagic...
Hereditary...
Hereditary spastic...
Hereditary spherocytosis
Hermansky-Pudlak syndrome
Hermaphroditism
Herpangina
Herpes zoster
Herpes zoster oticus
Herpetophobia
Heterophobia
Hiccups
Hidradenitis suppurativa
HIDS
Hip dysplasia
Hirschsprung's disease
Histoplasmosis
Hodgkin lymphoma
Hodgkin's disease
Hodophobia
Holocarboxylase...
Holoprosencephaly
Homocystinuria
Horner's syndrome
Horseshoe kidney
Howell-Evans syndrome
Human parvovirus B19...
Hunter syndrome
Huntington's disease
Hurler syndrome
Hutchinson Gilford...
Hutchinson-Gilford syndrome
Hydatidiform mole
Hydatidosis
Hydranencephaly
Hydrocephalus
Hydronephrosis
Hydrophobia
Hydrops fetalis
Hymenolepiasis
Hyperaldosteronism
Hyperammonemia
Hyperandrogenism
Hyperbilirubinemia
Hypercalcemia
Hypercholesterolemia
Hyperchylomicronemia
Hypereosinophilic syndrome
Hyperhidrosis
Hyperimmunoglobinemia D...
Hyperkalemia
Hyperkalemic periodic...
Hyperlipoproteinemia
Hyperlipoproteinemia type I
Hyperlipoproteinemia type II
Hyperlipoproteinemia type...
Hyperlipoproteinemia type IV
Hyperlipoproteinemia type V
Hyperlysinemia
Hyperparathyroidism
Hyperprolactinemia
Hyperreflexia
Hypertension
Hypertensive retinopathy
Hyperthermia
Hyperthyroidism
Hypertrophic cardiomyopathy
Hypoaldosteronism
Hypocalcemia
Hypochondrogenesis
Hypochondroplasia
Hypoglycemia
Hypogonadism
Hypokalemia
Hypokalemic periodic...
Hypoparathyroidism
Hypophosphatasia
Hypopituitarism
Hypoplastic left heart...
Hypoprothrombinemia
Hypothalamic dysfunction
Hypothermia
Hypothyroidism
Hypoxia
I
J
K
L
M
N
O
P
Q
R
S
T
U
V
W
X
Y
Z
Medicines

Diagnosis

Abnormal white blood cells bearing hair-like projections are seen on blood film examination, hence the name. Indicators include infection, anemia, and easy bleeding. Some of the leukemia cells may gather in the spleen and cause it to swell.

Treatment

It is treated with a form of chemotherapy on an outpatient basis. The drug is administered by a pump worn by the patient that provides regular injections over a 7 day period. During the weeks following treatment the patient's immune system is severely weakened, but his bone marrow will begin to produce normal white blood cells again. Treatment often results in long-term remission. If the cancer cells return, the treatment may be repeated and should again result in remission.

Read more at Wikipedia.org


[List your site here Free!]


Hairy cell leukemia with marked lymphocytosis
From Archives of Pathology & Laboratory Medicine, 2/1/03 by Adley, Brian Patrick

Hairy cell leukemia (HCL) is a rare small B-cell lymphoproliferative disorder. The neoplastic cells have round to oval nuclei and abundant cytoplasm with "hairy" projections seen in the peripheral blood and bone marrow. They typically diffusely infiltrate bone marrow and spleen. Immunophenotypically, they strongly express CD103, CD22, CD11c, and CD25. Hairy cell leukemia commonly presents with pancytopenia and splenomegaly with few circulating neoplastic cells. We describe the case of a 42-year-old man who presented at our institution with marked leukocytosis (white blood cell count, 98 300/(mu)L) and splenomegaly.

The patient had no significant past medical history. His hemoglobin level was 9.9 g/dL and his platelet count was 154000/(mu)L. Review of a peripheral blood smear demonstrated marked lymphocytosis consisting of larger than usual lymphocytes with round to oval, eccentrically located nuclei; small inconspicuous nucleoli; and relatively abundant cytoplasm (Figure 1). Most of the lymphocytes displayed cytoplasmic projections (Figure 1, inset). Many of the cells were tartrate-resistant acid phosphatase (TRAP) positive (Figure 2). A normochromic, normocytic anemia with occasional ovalocytes and teardrop cells was also noted. The bone marrow core biopsy was markedly hypercellular (90%), with most of the medullary space occupied by small to medium-sized, monotonous-appearing lymphocytes with abundant cytoplasm (Figure 3). Flow cytometric analysis performed on the bone marrow aspirate demonstrated these cells to be K-light chain restricted and CD19+, CD20+, CD5-, CD10-, CDllc+, CD103+, and CD25+.

The differential diagnosis of the peripheral blood smear in this case would include HCL; chronic lymphocytic leukemia; prolymphocytic leukemia; the leukemic phase of mantle cell, follicular, and marginal zone lymphomas; and the rare Japanese variant of HCL (HCLV). The morphology, cytochemistry, and immunophenotype (CD19+, CD20+, CDllc+, and CD103+) of the neoplastic cells in our case are diagnostic for classic HCL, although the high lymphocyte count is a very unusual finding. The HCL variant, although extremely uncommon, does typically present with a marked lymphocytosis (usually around 50000/(mu)L). However, the neoplastic cells in HCLV typically have prominent nucleoli (resembling prolymphocytes) and are CD25- and CD103-. This was not the case in our patient.

Cases of HCL with profound lymphocytosis have been reported only rarely in the literature. The highest white blood cell count reported was greater than 500 000/(mu)L, in a 49-year-old man who was treated with leukophoresis to bring his count to 20 000/(mu)L.1 Although the case was reportedly TRAP positive, immunophenotyping was not performed. A 71-year-old man with classic HCL (CD22+, CD25+ by immunohistochemistry) reportedly had a white blood cell count as high as 323 000/ lL. He eventually died as a result of leukostasis in the cerebral vasculature, which caused massive intracerebral hemorrhage.2 Golomb et al3 reviewed 71 cases of HCL and found that only 3 (4%) had white blood cell counts greater than 25 x 10^sup 3^/(mu)L. Other authors have reported similar results; however, in most cases immunophenotyping was not performed.

Our case represents another unusual case of HCL, with the diagnosis confirmed by morphology, immunophenotyping, and cytochemistry. Additionally, although the other cases cited were reportedly TRAP positive, our case may be the first reported in the literature with such a marked lymphocytosis (95 000/ (mu)L) that was shown to be CD11c+ and CD103+ (a relatively new and more specific antibody) by flow cytometry. Because positive TRAP staining is occasionally seen in other conditions, the question is raised as to whether some of the cases reported were truly cases of classic HCL. Moreover, this case as well as possibly the previous cases illustrate that although patients with classic HCL usually present with pancytopenia, rare cases can present with marked leukocytosis. This possibility should be kept in mind in order to provide accurate diagnosis and proper treatment of this disease. Our patient received a cycle of 2-chlorodeoxyadenosine (2CdA) immediately following diagnosis. Three months later, circulating hairy cells were not seen on peripheral blood smear. Platelet and white blood cell counts normalized. A bone marrow biopsy performed at this time showed minimal residual disease (approximately 5% hairy cells), and flow cytometry detected a small CD11c+, CD25+, CD103+ population of hairy cells. Currently, the patient is doing well clinically.

References

1. Worsley A, Cuttner J, Gordon R, Reilly M, Ambinder EP, Conjalka M. Therapeutic leukapheresis in a patient with hairy cell leukemia presenting with a white cell count greater than 500,000/microliter. Transfusion. 1982;22:308-310.

2. Ng MH, Tsang SS, Ng HK, Sriskandavarman V, Feng CS. An unusual case of hairy cell leukemia: death due to leukostasis and intracerebral hemorrhage. Hum Pathol. 1991;22:1298-1302.

3. Golomb HM, Catovsky D, Golde DW. Hairy cell leukemia: a clinical review based on 71 cases. Ann Internal Med. 1978;89:677-683.

Brian Patrick Adley, MD; Xiaoping Sun, MD, PhD; John M. Shaw, MD; Daina Variakojis, MD

Accepted for publication September 11, 2002.

From Northwestern University, Chicago, III (Drs Adley, Sun, and Variakojis); and Northwestern Memorial Hospital, Chicago, III (Dr Shaw).

Reprints: Brian P. Adley, MD, 251 E Huron, Feinberg Bldg 7-325, Chicago, IL 60611 (e-mail: b-adley@northwestern.edu).

Copyright College of American Pathologists Feb 2003
Provided by ProQuest Information and Learning Company. All rights Reserved

Return to Hairy cell leukemia
Home Contact Resources Exchange Links ebay