Neutropenia- severe chronic
Overview
A rare blood disorder where there are abnormally low levels of neutrophils (type of white blood cell) in the body which impairs the body's ability to fight bacterial infections. There are four main types of the disorder: idiopathic, congenital, autoimmune and cyclical
Symptoms
The list of signs and symptoms mentioned in various sources for Neutropenia, severe chronic includes the 4 symptoms listed below: * Recurring fevers * Mouth sores * Periodontitis * Recurring infections
Causes
Causes can be divided into the following groups: * Decreased production in the bone marrow: o aplastic anemia o cancer, particularly blood cancers o certain medications o hereditary disorders (e.g. congenital neutropenia, cyclic neutropenia) o radiation o Vitamin B12 or folate deficiency. * Increased destruction: o autoimmune neutropenia. o chemotherapy treatments, such as for cancer and autoimmune diseases * Marginalisation and sequestration: o Hemodialysis There is often a mild neutropenia in viral infections.
Diagnosis
Extrinsic to bone marrow o Acute infection –Viral (HAV, HBV, VZV, RSV, EBV) –Bacterial (group B strep, typhoid, TB, tularemia), fungal –Rickettsial (typhus, RMSF) –Protozoal (malaria, toxoplasmosis) * Drug-induced –Penicillin, sulfonamides –Ibuprofen, indomethacin –Ranitidine, cimetidine –Penicillamine –Barbiturates, benzodiazepines –Phenothiazines –Antithyroid medications –Anticonvulsants * Environmental toxins (arsenic, benzene) * Autoimmune * Isoimmune neonatal o Splenic or hepatic sequestration –Especially with concomitant mild thrombocytopenia or anemia o Metabolic disorders –Glycogen storage diseases Ib * Intrinsic to bone marrow or myeloid cell progenitors Chronic benign or idiopathic neutropenia * Cyclic neutropenia (autosomal dominant) * Marrow replacement with leukemia, lymphoma, or metastatic solid tumors * Kostmann syndrome –Severe congenital neutropenia * Hypo- or dysgammaglobulinemia * Myelodysplastic syndrome * Myelofibrosis * Schwachman syndrome o Fanconi anemia –May involve neutropenia, anemia, thrombocytopenia, or pancytopenia –Associated with absent radius, thumb abnormalities, short stature * Cartilage-hair hypoplasia * Dyskeratosis congenita * Chédiak-Higashi * Reticular dysgenesis * Myelokathexis
Treatment
The list of treatments mentioned in various sources for Neutropenia, severe chronic includes the following list. Always seek professional medical advice about any treatment or change in treatment plans. * Granulocyte colony stimulating factors