Leukopenia

Leukopenia exists when the total WBC count is less than 4000/mm3. Leukopenia may result from a decrease in one or more specific classes of leukocytes. The causes of neutropenia are listed in Table 9-6, lymphopenia in Table 9-14, and monocytopenia in Table 9-2. Leukopenia can result from a number of conditions. However,

Table 9-1. Causes of Leukocytosis

Physiologic

Newborn (maximal 38,000/mm3) Strenuous exercise Emotional disorders; fear, agitation Ovulation, labor, pregnancy Acute infections

Bacterial, viral, fungal, protozoal, spirochetal Metabolic causes Diabetic coma Acidosis Anoxia Azotemia Thyroid storm Acute gout Burns Seizures Drugs Steroids Epinephrine Endotoxin Lithium

Serotonin, histamine, heparin, acetylcholine Poisoning

Lead, mercury, camphor

Table 9-2. Causes of Monocytosis and Monocytopenia

Monocytosis

Hematologic disorders Leukemia

Acute myelogenous leukemia Chronic myelogenous leukemia Lymphoma (Hodgkin and non-Hodgkin) Chronic neutropenia Histiocytic medullary reticulosis Connective tissue disorders Systemic lupus erythematosus Rheumatoid arthritis Myositis Granulomatous diseases Inflammatory bowel disease Sarcoidosis Infections

Subacute bacterial endocarditis

Tuberculosis

Syphilis

Rocky Mountain spotted fever Kala-azar

Malignant disease (usually carcinomas) Miscellaneous disorders Postsplenectomy state Tetrachloroethane poisoning Lipidoses (e.g., Niemann-Pick disease)

Monocytopenia

Glucocorticoid administration Infections associated with endotoxemia

Acute hemorrhage Malignant neoplasms Carcinoma Sarcoma Lymphoma Connective tissue diseases Rheumatic fever Rheumatoid arthritis Inflammatory bowel disease Hematologic diseases

Splenectomy, functional asplenia Leukemia and myeloproliferative disorders Hemolytic anemia

Transfusion reaction Megaloblastic anemia during therapy

Table 9-3. Causes of Basophilia

Hypersensitivity reactions

Drug and food hypersensitivity Urticaria Inflammation and infection Ulcerative colitis Rheumatoid arthritis Influenza Chickenpox Smallpox Tuberculosis Myeloproliferative diseases

Chronic myeloid leukemia Myeloid metaplasia

Table 9-4. Causes of Neutrophilia

Increased production

Clonal disease

Myeloproliferative disorders Chronic myelogenous leukemia Chronic neutrophilic leukemia Juvenile myelomonocytic leukemia

Transient myeloproliferative disorder of Down syndrome Hereditary

Autosomal dominant form of hereditary neutrophilia Familial cold urticaria Reactive

Chronic infection Chronic inflammation Juvenile rheumatoid arthritis Inflammatory bowel disease Kawasaki disease Hodgkin disease

Drugs: lithium, G-CSF, GM-CSF, chronic use of corticosteroids

Leukemoid reaction

Chronic idiopathic neutrophilia

Increased mobilization from marrow storage pool

Drugs: Corticosteroids, G-CSF Stress

Acute infection Hypoxia

Decreased margination

Exercise Epinephrine

Decreased egress from circulation

Leukocyte adhesion deficiency (LAD)

LAD type I: deficiency of CD 11/CD 18 integrins on leukocytes LAD type II: absence of neutrophil sialyl Lewis X structures Asplenia

Modified from Dinaur MC. The phagocyte system and disorders of granulopoiesis and granulocyte function. In: Nathan and Oski's Hematology of Infancy and Childhood. 5th ed. Philadelphia: WB Saunders, 1998, with permission.

Table 9-5. Features of Leukemoid Reaction and Leukemia

Feature Leukemoid reaction Leukemia

Clinical Evidence of infection Hepatosplenomegaly

Lymphadenopathy Hematologic No anemia Anemia

No thrombocytopenia Thrombocytopenia

Bone marrow Normal, hypercellular Blasts

Decreased megakaryocytes Decreased erythroid precursors Leukocyte alkaline High Absent phosphatase

Table 9-6. Causes of Neutropenia

I. Decreased production

A. Congenital

1. Neutropenia in various ethnic groups"

2. Hereditary a. Severe congenital neutropenia: sporadic (most common) or autosomal dominant or Kostmann disease—autosomal recessive b. Familial benign chronic neutropenia—autosomal dominant

3. Chronic benign neutropenia6

4. Reticular dysgenesis

5. Cyclic neutropenia

6. Neutropenia associated with agammaglobulinemia and dysgammaglobulinemia

7. Neutropenia associated with abnormal cellular immunity in cartilage-hair hypoplasia

8. Neutropenia associated with pancreatic insufficiency [Shwachman-Diamond syndrome (see page 221) and Pearson syndrome (see page 126); (Chapter 6; Table 6-24)]

9. Neutropenia associated with hyperimmunoglobulin M syndrome

10. Neutropenia associated with metabolic disease a. Glycogen storage disease (type IB)

b. Idiopathic hyperglycinemia c. Isovaleric acidemia d. Methylmalonic acidemia e. Propionic acidemia f. Thiamine-responsive anemia in DIDMOAD syndrome (see pages 64-65)

g. Barth syndromec (see page 223)

11. Bone marrow aplasia a. Fanconi anemia b. Familial congenital aplastic anemia without anomalies c. Dyskeratosis congenita (see pages 112-114) (Chapter 6)

12. Bone marrow infiltration: osteopetrosis, cystinosis, Gaucher disease, Niemann-Pick disease

B. Acquired

1. Acute a. Acute transient neutropenia b. Viral infection (e.g., HIV, EBV, hepatitis A and B, respiratory syncytial virus, measles, rubella, varicella)

c. Bacterial infection (e.g., typhoid, paratyphoid, tuberculosis, brucellosis)

d. Rickettsial infection

Table 9-6. (Continued)

2. Chronic a. Bone marrow aplasia

(1) Idiopathic

(2) Secondary: drugs, chemicals, irradiation, infection, immune reaction, malnutrition, copper deficiency, vitamin B12 deficiency, folate deficiency b. Bone marrow infiltration, neoplastic

(1) Primary: leukemia

(2) Secondary: neuroblastoma, lymphoma, rhabdomyosarcoma

II. Failure to release mature neutrophils from the bone marrow (myelokathexis)

(ineffective myelopoiesis) Cortisone stimulation test (Table 9-8)

III. Increased margination of neutrophils (pseudoneutropenia)

Epinephrine stimulation test (Table 9-8)

IV. Increased destruction

A. Immune

1. Drug induced (e.g., anticonvulsants)

2. Alloimmune (isoimmune)

a. Maternofetal b. Multitransfusion

3. Autoimmune neutropenia a. Idiopathica b. Secondary: systemic lupus erythematosus, lymphoma, leukemia, rheumatoid arthritis, HIV infection (in 20-44% of AIDS patients), infectious mononucleosis, associated with autoimmune thrombocytopenia and/or autoimmune hemolytic anemia

B. Nonimmune

1. Infections

2. Hypersplenism

"Chronic, mild with a benign course.

"Probably same condition as idiopathic autoimmune neutropenia.

cRare genetic disorder consisting of neutropenia, cardiomyopathy, muscle weakness, failure to thrive and growth retardation.

isolated leukopenia resulting from a decrease in all classes of leukocytes is observed uncommonly.

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Responses

  • kalevi
    What is leukopenia may be a result from?
    6 years ago
  • mauro
    Is chronic benign leukopenia a genetic disorder?
    6 years ago

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