Granulocytopenia and lymphocytopenia
Granulocytopenia and lymphocytopenia: Excerpt from Professional Guide to Diseases (Eighth Edition)
Granulocytopenia (also called agranulocytosis) is characterized by a marked reduction in the number of circulating granulocytes. Although this implies all the granulocytes (neutrophils, basophils, and eosinophils) are reduced, granulocytopenia usually refers to decreased neutrophils. (See Understanding neutropenia.) 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. The severest form is known as agranulocytosis.
Lymphocytopenia (sometimes called lymphopenia), a rare disorder, is a deficiency of circulating lymphocytes (leukocytes produced mainly in lymph nodes).
In both granulocytopenia and lymphocytopenia, the total leukocyte count (white blood cell [WBC] count) may reach dangerously low levels, 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 may result from diminished production of granulocytes in bone marrow, increased peripheral destruction of granulocytes, or greater utilization of granulocytes. Diminished 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 phenothiazine, sulfonamides (and some sulfonamide derivatives), antibiotics, and antiarrhythmic drugs. Drug-induced granulocytopenia usually develops slowly and typically correlates with the dosage and duration of therapy. Production of granulocytes also decreases in conditions such as aplastic anemia and bone marrow malignancies and in some hereditary disorders (infantile genetic agranulocytosis).
The growing 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.
Similarly, lymphocytopenia may result from decreased production, increased destruction, or loss of lymphocytes. Decreased production of lymphocytes may be secondary to a genetic or a thymic abnormality or to immunodeficiency disorders, such as thymic dysplasia or ataxia-telangiectasia. Increased destruction of lymphocytes may be secondary to radiation, chemotherapy, or human immunodeficiency virus infection. Loss of lymphocytes may follow postsurgical thoracic duct drainage, intestinal lymphangiectasia, or 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, or heart failure). Other disorders associated with lymphocyte depletion 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
Characteristically, patients with granulocytopenia experience slowly progressive fatigue and weakness; however, if they develop an infection, they can exhibit sudden onset of fever and chills and mental status changes. Overt signs of infection (pus formation) are usually absent. Localized infection can quickly become systemic (bacteremic) or spread throughout an organ (pneumonia). All patients should be meticulously evaluated for even subtle signs of infection because untreated infections can lead to septic shock in 8 to 24 hours. If granulocytopenia results from 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
Diagnosis of granulocytopenia necessitates a thorough patient history to check for precipitating factors. Physical examination for clinical effects of underlying disorders is also essential.
Confirming diagnosis A markedly decreased neutrophil count (less than 500/µl leads to severe bacterial infections) and a WBC count lower than 2,000/µl, with few observable granulocytes on complete blood count (CBC), confirm granulocytopenia.
Bone marrow examination shows a scarcity of granulocytic precursor cells beyond the most immature forms, but this may vary, depending on the cause.
A lymphocyte count below 1,500/µl in adults or below 3,000/µl in children indicates lymphocytopenia. Identifying the cause by evaluation of clinical status, bone marrow and lymph node biopsies, or other appropriate diagnostic tests helps establish the diagnosis.
Treatment
Effective management of granulocytopenia must identify and eliminate the cause and control infection until the bone marrow can generate more leukocytes. In many cases, this means 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 granulocyte colony-stimulating factor (CSF) or granulocyte-macrophage CSF is a newer 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, mouth, nose, rectum, vagina, and sputum, as ordered. Give antibiotics as scheduled.
❑ Explain the necessity of infection protection procedures to the patient and his family. Teach proper hand-washing technique and the correct use of gowns and masks. Prevent patient contact with staff or visitors with respiratory infections.
❑ Maintain adequate nutrition and hydration because malnutrition aggravates immunosuppression. Make sure the patient with mouth ulcerations receives a high-calorie liquid diet. Offer a straw to make drinking less painful.
❑ Provide warm saline water gargles and rinses, analgesics, and anesthetic lozenges. Good oral hygiene promotes comfort and facilitates healing.
❑ 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.
❑ To help detect granulocytopenia and lymphocytopenia in the early, most treatable stages, monitor the WBC count of any patient receiving radiation 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.
Pictures
Book Source Details
- Book Title: Professional Guide to Diseases (Eighth Edition)
- Author(s): Springhouse
- Year of Publication: 2005
- Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.
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