Post on 27-May-2015
description
Congenital Causes
- Infantile agranulocytosis- Shwachman-Diamond syndrome- Chediak-Higashi syndrome- Reticular dysgenesis.
Acquired causes Post-infectious - bacterial, parasitic, rickettsial,
and viral infections. Drug-induced neutropenia Chronic idiopathic neutropenia Autoimmune neutropenia Collagen/vascular disorders like SLE and RA Deficiencies of dietary vitamins and minerals
(eg, vitamin B12, folate, copper) Myelodysplastic syndromes and hematologic
malignancies Aplastic anemia Paroxysmal nocturnal hemoglobinuria
T- Cell LGL Indolent leukemia comprising of 2% to 5% of the chronic
lymphoproliferative diseases. It usually presents around 60 years, equally affecting males
and females. Disorders like rheumatoid arthritis can be associated with
LGL leukemia in 11 to 36% of the cases. There is no standard treatment for patients with LGL
leukemia. Therapy is recommended in patients with ANC < 500 or
those with ANC > 500 and recurrent symptomatic infections. Asymptomatic patients may observed and treated with
supportive measures like G-CSF as needed. Immunosuppressive therapy is the definitive treatment in
the current era, with agents like methotrexate, oral cyclophosphamide, and cyclosporine.
Cocaine induced neutropenia
Occurs due to contamination with levamisole.
Causes immune mediated destruction of the neutrophils .
Improves with cocaine abstinence.
Testing for levamisole, if needed can be done in plasma and urine.
Plasma half-life of levamisole is relatively short (5.6 h), serum testing is likely to be negative if a patient’s last exposure occurred more than 24 h. Urine testing will be positive if done within 2-3 days.
References Buchanan JA, Lavonas EJ. Agranulocytosis and other consequences
due to use of illicit cocaine contaminated with levamisole. Curr Opin Hematol. 2012;19(1):27-31.
Zhang D, Loughran TP Jr. Large granular lymphocytic leukemia: molecular pathogenesis, clinical manifestations, and treatment. Hematology Am Soc Hematol Educ Program. 2012;2012:652-9.