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Neutropenia

Author/s: 
Mithoowani, Siraj, Cameron, L., Crowther, M. A.

»Neutropenia is seen in 5%–10% of healthy people
Based on absolute neutrophil count, neutropenia is commonly defined as
mild (1.0–1.5 × 109
/L), moderate (0.5–0.9 × 109
/L) or severe (< 0.5 × 109
/L).1
However, the reference interval is specific to the population. Mild asymptomatic neutropenia per the above definition is common in people of subSaharan African, Arab or West Indian ancestry,2
and is strongly associated
with the Duffy-null phenotype of red blood cells that protects against
Plasmodium vivax malaria.
2 Common causes include medications, infection, nutritional
deficiency, malignant disease and autoimmune disease
Causes include underproduction (e.g., myelodysplastic syndrome),
immune-mediated destruction or redistribution of neutrophils to the
endothelium and reticuloendothelial system. Antithyroid, anti-infective
and psychotropic drugs, as well as chemotherapy, are causes of druginduced neutropenia.3,4 Transient neutropenia may occur after acute
viral infection and typically resolves within 2 weeks. Joint swelling, rash,
bony pain, splenomegaly or lymphadenopathy may suggest malignant or
autoimmune disease.
3Investigation should begin with a repeat complete blood count
and peripheral blood film
Neutropenia is often identified incidentally. Persistent and unexplained
neutropenia requires further work-up for a range of causes, including
chronic viral infection (e.g., hepatitis, HIV) and nutritional deficiency (e.g.,
vitamin B12) (Appendix 1, available at www.cmaj.ca/lookup/doi/10.1503/
cmaj.220499/tab-related-content).
4 Treatment of mild neutropenia should be directed at the
underlying cause
Patients with mild neutropenia are not at substantially increased risk of
infection.5
The neutrophil count should be checked every 3–6 months for
at least 1 year to rule out progression to more severe neutropenia.
5 Patients with moderate-to-severe neutropenia for more than
6 months should be referred to a specialist
Patients with recurrent or severe bacterial infections (e.g., requiring hospital admission or intravenous antibiotics), abnormalities on peripheral blood
films (e.g., circulating blasts, hairy cells, large granular lymphocytes, dysplastic granulocytes) or pancytopenia also warrant referral to a specialist
(e.g., hematologist, internist, pediatrician).1
Febrile neutropenia (absolute
neutrophil count < 0.5 × 109
/L and an oral temperature > 38.0°C sustained
over 1 h) requires immediate treatment with broad-spectrum antibiotics.

Keywords 
neutropenia complete blood count neutrophil count treatment Autoimmune Diseases

Diagnosis and Treatment of Myelodysplastic Syndromes: A Review

Author/s: 
Sekeres, M. A., Taylor, J.

Importance Myelodysplastic neoplasms (MDS), formerly known as myelodysplastic syndromes, are clonal hematopoietic malignancies that cause morphologic bone marrow dysplasia along with anemia, neutropenia, or thrombocytopenia. MDS are associated with an increased risk of acute myeloid leukemia (AML). The yearly incidence of MDS is approximately 4 per 100 000 people in the United States and is higher among patients with advanced age.

Observations MDS are characterized by reduced numbers of peripheral blood cells, an increased risk of acute myeloid leukemia transformation, and reduced survival. The median age at diagnosis is approximately 70 years, and the yearly incidence rate increases to 25 per 100 000 in people aged 65 years and older. Risk factors associated with MDS include older age and prior exposures to toxins such as chemotherapy or radiation therapy. MDS are more common in men compared with women (with yearly incidence rates of approximately 5.4 vs 2.9 per 100 000). MDS typically has an insidious presentation, consisting of signs and symptoms associated with anemia, thrombocytopenia, and neutropenia. MDS can be categorized into subtypes that are associated with lower or higher risk for acute myeloid leukemia transformation and that help with therapy selection. Patients with lower-risk MDS have a median survival of approximately 3 to 10 years, whereas patients with higher-risk disease have a median survival of less than 3 years. Therapy for lower-risk MDS is selected based on whether the primary clinical characteristic is anemia, thrombocytopenia, or neutropenia. Management focuses on treating symptoms and reducing the number of required transfusions in patients with low-risk disease. For patients with lower-risk MDS, erythropoiesis stimulating agents, such as recombinant humanized erythropoietin or the longer-acting erythropoietin, darbepoetin alfa, can improve anemia in 15% to 40% of patients for a median of 8 to 23 months. For those with higher-risk MDS, hypomethylating agents such as azacitidine, decitabine, or decitabine/cedazuridine are first-line therapy. Hematopoietic cell transplantation is considered for higher-risk patients and represents the only potential cure.

Conclusions and Relevance MDS are diagnosed in approximately 4 per 100 000 people in the United States and are associated with a 5-year survival rate of approximately 37%. Treatments are tailored to the patient’s disease characteristics and comorbidities and range from supportive care with or without erythropoiesis-stimulating agents for patients with low-risk MDS to hypomethylating agents, such as azacitidine or decitabine, for patients with higher-risk MDS. Hematopoietic cell transplantation is potentially curative and should be considered for patients with higher-risk MDS at the time of diagnosis.

Keywords 
Leukemia Hematinics Prognosis Myedysplastic syndromes drug therapy
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