Neutropenia

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What is neutropenia?

Before discussing what neutropenia IS, we need to talk about what a neutrophil is!  Neutrophils are a type of white blood cell (leukocytes) called granulocytes.  Neutrophils kill bacteria/pathogens by engulfing them (phagocytosis).  Neutrophils are produced in the bone marrow along with other blood cells.  For this discussion, we will only talk about neutrophils.  The mature neutrophils leave the bone marrow and enter the blood stream (peripheral blood).   A neutrophil has a lifespan in the bloodstream of 6-10 hours.

Neutropenia is a decrease in circulating neutrophils in the peripheral blood. The absolute neutrophil count (ANC) defines neutropenia. ANC is found by multiplying the percentage of bands and neutrophils (relative count) by the total white blood cell count. The CBC differential count measures the types of white blood cells (WBCs) as a percentage of the total WBC count, this is the relative count.  To obtain the Absolute count, one must multiply the relative value of each type of cell (neutrophils in our case) by the total WBC count. If a person has a total WBC of 6,000 and his differential shows 30% neutrophils, to figure the Absolute Neutrophil Count (ANC) we multiply 6,000 by 30% to arrive at an ANC of 1800. 

The severity of neutropenia is categorized as mild with an ANC of 1000-1500 , moderate with an ANC of 500-1000 , and severe with an ANC of lower than 500 . The risk of bacterial infection is related to the severity and duration of neutropenia.  Sources vary on what they consider neutropenia to be.  Some textbooks refer to anything below an ANC of 2,000 to be neutropenic and others use an ANC of 1,800.   An ANC of 2,000 is considered to be  within the normal range, but keep in mind that the average person has an ANC in the 3,000 range.  Everyone has a cycle and in most normal folks the ANC doesn't drop below 2,000.  Viruses and bacterial infections can affect neutrophil counts in anyone.

How many people are affected by neutropenia?

An e-medicine article by Dr. Daniel Shin gives the following occurence rate for neutropenia.  Internationally: The incidence of agranulocytosis is 3.4 cases per million persons per year. The incidence of drug-induced neutropenia is 1 case per million persons per year.
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What are the causes of neutropenia?

There are many types of neutropenia.  Cancer patients often have drug induced neutropenia and will receive a drug called G-CSF to help boost their counts. Nathan and Oski's Hematology of Infancy and Childhood 5th Edition classifies the types of neutropenia as follows. 

Neutropenia caused by Intrinsic defects in myeloid Cells or their progenetors:

  • Reticular dysgenesis
  • Severe congenital neutropenia (Kostmann's Syndrome)
  • Cyclic neutropenia
  • Myelokathexis
  • Shwachman-Diamond Syndrome
  • Dyskeratosis congenita
  • Chediak-Higashi syndrome
  • Famililial benign neutropenia
  • Fanconi's anemia
  • Bone Marrow Failure syndromes

 Neutropenia caused by extrinsic factors

  •  infection
  • drug-induced neutropenia
  •  chronic benign neutropenia of childhood (autoimmune neutropenia of childhood)
  •  immune neonatal neutropenia
  •  neutropenia associted with immune dysfunction
  •  neutropenia associated with metabolic diseases
  •  nutritional deficiencies
  •  reticuloendotheial sequestration
  •  bone marrow infiltration
  •  chronic idiopathic neutropenia.

When someone has neutropenia, what can happen?

People who have neutropenia are at risk of infection.  When a person has an ANC below 500, they are at risk from infection from the bacteria on their own bodies!  People with neutropenia often get mouth sores and have dental problems.  From our own experience, Sean lost a tooth five years too early due to reabsorption. We have been told that reabsorption is something that can occur in people with leukopenia/neutropenia.  The tooth was literally eaten from within.  It looked normal from the outside, so until he had problems we had no idea there was a problem.  A year later, Joseph had the same problem with one of his molars!Proper oral hygeine is important for anyone with neutropenia. 

Various sources list precautions to be taken by someone who has neutropenia.  For more information, please see the information links.  Yahoogroups has a wonderful neutropenia support group/email list.  If you would like to become a member, send an email to neutropenia-subscribe@yahoogroups.com

 

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