Granulocytopenia and lymphocytopenia
Granulocytopenia and lymphocytopenia: Excerpt from Handbook of Diseases
In granulocytopenia, a marked reduction in the number of circulating granulocytes occurs. Although this implies that all the granulocytes (neutrophils, basophils, and eosinophils) are reduced, granulocytopenia usually refers only to decreased neutrophils.
This disorder, which can occur at any age, is associated with infections and ulcerative lesions of the throat, GI tract, other mucous membranes, and skin. Its most severe form is known as agranulocytosis.
Lymphocytopenia (lymphopenia), a rare disorder, is a deficiency of circulating lymphocytes (leukocytes produced mainly in lymph nodes).
In granulocytopenia and lymphocytopenia, the total white blood cell (WBC) count may reach dangerously low levels (less than 500/µl), leaving the body unprotected against infection. The prognosis in both disorders depends on the underlying cause and whether it can be treated. Untreated, severe granulocytopenia can be fatal in 3 to 6 days.
Causes
Granulocytopenia and lymphocytopenia have several causes.
Granulocytopenia
Granulocytopenia may result from decreased production of granulocytes in bone marrow, increased peripheral destruction of granulocytes, or greater utilization of granulocytes.
Decreased production of granulocytes in bone marrow generally stems from radiation or drug therapy; it’s a common adverse effect of antimetabolites and alkylating agents and may occur in the patient who is hypersensitive to phenothiazines, sulfonamides (and some sulfonamide derivatives such as chlorothiazide), antibiotics, or antiarrhythmic drugs.
Drug-induced granulocytopenia usually develops slowly and typically correlates with the dosage and duration of therapy. Production of granulocytes also decreases in such conditions as aplastic anemia and bone marrow cancers and in some hereditary disorders such as infantile genetic agranulocytosis.
Loss of peripheral granulocytes is due to increased splenic sequestration, diseases that destroy peripheral blood cells (viral and bacterial infections), and drugs that act as haptens (carrying antigens that attack blood cells and causing acute idiosyncratic or non-dose-related drug reactions).
Infections, such as infectious mononucleosis, may result in granulocytopenia because of increased utilization of granulocytes.
Lymphocytopenia
Similarly, lymphocytopenia may result from the decreased production, increased destruction, or loss of lymphocytes. Decreased production of lymphocytes may occur secondary to a genetic or thymic abnormality or to immunodeficiency disorders, such as ataxia-telangiectasia and thymic dysplasia. Increased destruction of lymphocytes may occur secondary to radiation therapy or chemotherapy (with alkylating agents).
Loss of lymphocytes may follow postsurgical thoracic duct drainage, intestinal lymphangiectasia, and impaired intestinal lymphatic drainage (as in Whipple’s disease). Lymphocyte depletion can also result from elevated plasma corticoid levels (due to stress, corticotropin or steroid treatment, and heart failure).
Other associated disorders include Hodgkin’s disease, leukemia, aplastic anemia, sarcoidosis, myasthenia gravis, lupus erythematosus, protein-calorie malnutrition, renal failure, terminal cancer, tuberculosis and, in infants, severe combined immunodeficiency disease (SCID).
Signs and symptoms
Patients with granulocytopenia typically experience slowly progressive fatigue and weakness, followed by the sudden onset of signs of overwhelming infection (fever, chills, tachycardia, anxiety, headache, and extreme prostration); ulcers in the mouth or colon; pharyngeal ulceration, possibly with associated necrosis; pneumonia; and septicemia, possibly leading to septic shock and death.
If granulocytopenia is caused by an idiosyncratic drug reaction, signs of infection develop abruptly, without slowly progressive fatigue and weakness.
Patients with lymphocytopenia may exhibit enlarged lymph nodes, spleen, and tonsils and signs of an associated disease.
Diagnosis
Granulocytopenia is diagnosed by a thorough patient history to check for precipitating factors. Physical examination for clinical effects of underlying disorders is also essential. The following test results confirm the diagnosis:
❑ Complete blood count (CBC) reveals a marked reduction in neutrophils (less than 500/µl leads to severe bacterial infections) and a WBC count lower than 2,000/µl with few observable granulocytes.
❑ Examination of bone marrow generally shows a scarcity of granulocytic precursor cells beyond the most immature forms, but this finding may vary, depending on the cause.
❑ Lymphocyte count less than 1,500/µl in adults or less than 3,000/µl in children indicates lymphocytopenia. Identifying the cause by evaluation of the patient’s clinical status, bone marrow and lymph node biopsies, and other appropriate diagnostic tests helps establish the diagnosis.
Treatment
Effective management of granulocytopenia involves identification and elimination of the cause and controlling infection until the bone marrow can generate more leukocytes. In many cases, this means that drug or radiation therapy must be stopped and antibiotic treatment begun immediately, even while awaiting test results. Treatment may also include antifungal preparations.
Administration of a colony-stimulating factor, such as filgrastim or sargramostim, is another treatment used to stimulate bone marrow production of neutrophils. Spontaneous restoration of leukocyte production in bone marrow generally occurs within 1 to 3 weeks.
Treatment of lymphocytopenia includes eliminating the cause and managing any underlying disorders. For infants with SCID, therapy may include bone marrow transplantation.
Special considerations
❑ Monitor vital signs frequently. Obtain cultures from blood, throat, urine, and sputum. Give antibiotics as scheduled.
❑ Explain the necessity for protective isolation (preferably with laminar air flow) to the patient and his family.
❑ Teach proper hand-washing technique and how to correctly use gowns and masks. Prevent patient contact with staff members or visitors with respiratory tract infections.
❑ Maintain adequate nutrition and hydration because malnutrition aggravates immunosuppression. Make sure the patient with mouth ulcers receives a high-calorie, liquid diet — for example, high-protein milk shakes. Offer a straw to make drinking less painful.
❑ Provide warm saline water gargles and rinses, analgesics, and anesthetic lozenges because good oral hygiene promotes patient comfort and facilitates the healing process.
❑ Ensure adequate rest, which is essential to the mobilization of the body’s defenses against infection. Provide good skin and perineal care.
❑ Monitor CBC and differential, blood culture results, serum electrolyte levels, intake and output, and daily weight.
Clinical tip To help detect granulocytopenia and lymphocytopenia in the early, most treatable stages, monitor the WBC count of any patient receiving radiation therapy or chemotherapy. After the patient has developed bone marrow depression, he must zealously avoid exposure to infection.
❑ Advise the patient with known or suspected sensitivity to a drug that may lead to granulocytopenia or lymphocytopenia to alert medical personnel to this sensitivity in the future.
Book Source Details
- Book Title: Handbook of Diseases
- Author(s): Springhouse
- Year of Publication: 2003
- Copyright Details: Handbook of Diseases, Copyright © 2003 Lippincott Williams & Wilkins.
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More About This Book:
Title: Handbook of Diseases
Authors: Springhouse
Publisher: Lippincott Williams & Wilkins
Copyright: 2003
ISBN: 1-58255-266-5
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