Decreased # neutrophils <1500 mm3, defective function, diabetes mellitus, corticosteroid therapy, alcoholism, renal failure

Benign ethnic neutropenia seen w/ African and Middle Eastern ancestry

Idiopathic

Medications: anticancer, antibiotics-penicillin and sulfonamides, phenothliazines, tranquilizers, diuretics

VIral & bacterial infections

Autoimmune disease such as SLE - antibodies to neutrophil produced

Hereditary (infections in infancy): Schwachman-Diamond syndrome, Dyskeratosis congenita, Cartilage-hair syndrome, Severe congenital neutropenia

- Females>males, increases w/ increased age

- Bacterial infection (rather than fungal or viral) w/ staph aureus or other gram neg mo

- Fever

- Infections of middle ear, oral, perirectal

- <500/mm3 associated with pneumonia

- Oral-gingival ulcerations

Variable, good prognosis if responds to G-CSF

10-30% mortality

Cells of granulocytic line are absent (neutrophils, eosinophils, basophils)

Incidence 3.4 cases/million

Idiopathic

Medication

Immune rxns

Congenital syndrome: congenital agranulocytosis, Kostmann syndrome (decrease G-CSF)

Bacterial infection, ulcerations

Perioral and ear

Ulcers often lack erythema

Withdraw offending agent

Supportive therapy (CHX, antibiotics)

Meticulous oral hygiene

Granylocyte colony stimulating factor or granulocyte-macrophage colony stimulating factor

Guarded

20-30% mortality in past prior to antibiotic and cytokine therapy

Rare, hereditary or isolated

Regular periodic reduction in neutrophils (monocytes, lymphocytes, and platelets also cycle)

Autosomal dominant, defects in gene ELA2 (neutrophil elastase 2, also known as medullasin)

Male=female

susceptible to recurrent infections (SX at nadir, lowest point)

Symptoms begin in childhood: fever, maliase; mouth sores; gingivitis; alveolar bone loss; cycle is 18-21 days long

Blood tests: complete blood cell counts (CBC) 2-3xweekly for 8 wks; less than 500 mm3 for 3-5 days in at least 3 successive cycles

Biopsy: ulcer with few neutrophils

Supportive, CHX rinses 2x/day, antibiotics, meticulous OH

Human granulocyte-colony stimulating factor reduces time and duration of cycle, decreases severity of mucositis and infection

- CBCs remain the same, but the clinical signs diminsh after age 20 yrs

- Alveolar bone loss may be permenant

- Used animal model in collie, lethal (nose wasn't black)

- Increased #s of leukocytes

- Reactive or neoplastic: neutrophilic leukocytosis-bacterial infection or tissue necrosis; eosinophilia-parasitic infections, hypersensitivity rxns, Hodgkin's disease; lymphocytosis-viral infections; monocytosis-infective endocarditis, protozoal infections

EBV

Intimate contact required-saliva US 50% by age 18 yrs

Herpes virus fmaily- remains latent lifelong

- Rapid diagnosis kits-Monospot (Horner), Monosticon (Organon)

- Immunofluorescent serological testing for Paul Bunell heterophile antibody or ELISA or DNA based tests

CBC and differential: lymphocytosis w/ "atypical lymphocytes" 70-90%

- Malignant proliferation of stem cell in bone marrow

- Spills into peripheral circulation

Crowds out normal marrow --> clinical symptoms

13 cases/100,000/year

Slightly increased in males > females

- Combination of genetic & environmental factors

- Genetic: down syndrome, bloom syndrome, neurofibromatosis, Schwachman-Diamond syndrome, Ataxia-telangiectasia syndrome, Klinefelter syndrome, Fanconi's anemia, Wiskhott-Aldrich syndrome

- Chromosomal abnormalities (Phily chrom)

Environmental: pesticides, benzene; ionizinng radiation; viruses HTLV-1

Behavior: acute (blast cells) vs. chronic (mature cells)

Cell type: ALL acute lymphocytic leukemia - children

ANLL acute non-lymphocytic (adults); Hairy cell, responds to interferon

- Expands bone marrow & infiltrates organs and peripheral blood myelophthisic anemia

- Bleeding, bruising, fatigue, infections, fever

- Oral ulcers, petechiae, bleeding, swollen gingiva, mimics periodontal disease or periapical inflammatory disease

- Rare-greenish hyperplastic tissue mass-chloroma (granulocytic sarcoma or extramedullary myeloid tumor)

Peripheral blood

Bone marrow aspirate or biopsy

Biopsy of hyperplastic gingival lesions

Chemo, radiation to CNS or intrathecal administration

Gleevac (cancer drug)

Sprycel

Bone marrow transplant

Supportive transfusions, meticulous OH and dental care for infections

etiology: unknown

Preleukemic condition

Over 50 yrs of age

Quantitative and qualitative abnormalities of all cell lines

May be associated w/ non-necrotizing granulomatous inflammation