About granulocytopenia primary

What is granulocytopenia primary?

Acquired agranulocytosis is a rare, drug-induced blood disorder that is characterized by a severe reduction in the number of white blood cells (granulocytes) in the circulating blood. The name granulocyte refers to grain-like bodies within the cell. Granulocytes include basophils, eosinophils, and neutrophils.

Acquired agranulocytosis may be caused by a variety of drugs. However, among the drugs to which a patient may be sensitive are several used in the treatment of cancer (cancer chemotherapeutic agents) and others used as antipsychotic medications (e.g., clozapine). The symptoms of this disorder come about as the result of interference in the production of granulocytes in the bone marrow.

People with acquired agranulocytosis are susceptible to a variety of bacterial infections, usually caused by otherwise benign bacteria found in the body. Not infrequently, painful ulcers also develop in mucous membranes that line the mouth and/or the gastrointestinal tract.

What are the symptoms for granulocytopenia primary?

The first symptoms of acquired agranulocytosis are usually those associated with a bacterial infection such as general weakness, chills, fever, and/or extreme exhaustion. Symptoms that are associated with rapidly falling white blood cell levels (granulocytopenia) may include the development of infected ulcers in the mucous membranes that line the mouth, throat, and/or intestinal tract. Some people with these ulcers may experience Difficulty swallowing due to irritation and pain.

Granulocytopenia causes a concurrent decrease in the number of neutrophils in the circulating blood (neutropenia). As neutrophil levels decrease, the susceptibility of patients with acquired agranulocytosis to bacterial infections becomes even greater. Fevers and abnormal enlargement of the spleen (splenomegaly) are characteristic features of neutropenia. If neutropenia is not treated, bacterial infections can lead to life-threatening complications such as bacterial shock or bacterial contamination of the blood (sepsis.)

Chronic acquired agranulocytosis generally progresses more slowly than acquired agranulocytosis. Canker sores in the mouth and chronic Inflammation of the gums (gingivitis) may be recurring symptoms. Other systemic infections may recur regularly.

What are the causes for granulocytopenia primary?

Acquired agranulocytosis is almost invariably caused by exposure to drugs and/or chemicals. Any chemical or drug that depresses the activity of the bone marrow may cause agranulocytosis. Some drugs cause this reaction in anyone given large enough doses. Other drugs may cause the reaction in one person but not in another (idiosyncratic). Clinicians do not understand why some people are susceptible to agranulocytosis and others are not.

In some instances, the action of some drugs or chemicals suggests that the immune system is involved. In the case of gold, or anti-thyroid drugs, or quinidine, among others, antibodies are created that appear to break the granulocytes down.

Other drugs that interfere with, or inhibit, granulocyte colony formation may induce agranulocytosis. Drugs with this characteristic include valproic acid, carbamazepine, and the beta-lactam antibiotics.

A complicating factor is that several commonly used anti-cancer drugs are prone to cause agranulocytosis, thus interfering with treatment. The same may be said for several anti-psychotic medications.

A variety of drugs can cause acquired agranulocytosis and neutropenia by destroying special cells in the bone marrow that later mature and become granulocytes (precursors). These drugs include phenytoin, pyrimethamine, methotrexate, and cytarabine. In rare cases of acute acquired agranulocytosis, destructive action of certain white blood cell antibodies (leukocyte isoantibodies) may be induced by certain drugs such as phenylbutazone, gold salts, sulfapyridine, aminopyrine, meralluride, and dipyrine.

What are the treatments for granulocytopenia primary?

Filgrastim (Neupogen) has been designated an orphan drug and approved by the U.S. Food and Drug Administration (FDA) for the treatment of severe, chronic neutropenia; and it has become a standard treatment for acquired agranulocytosis. Filgrastim is one of a class of colony-stimulating factors that does, indeed, stimulate the proliferation and differentiation of neutrophils. It is manufactured by Amgen, Inc., using recombinant DNA technology.

The treatment of acquired agranulocytosis includes the identification and elimination of drugs or other agents that induce this disorder. Antibiotic medications may also be prescribed if there is a positive blood culture for the presence of bacteria or if a significant local infection develops.

Treatment in adults with antibiotics should be limited to about 7-10 days since longer duration carries with it a greater risk of kidney (renal) complications and may set the stage for a new infection. When granulocyte levels return to a near normal range, fever and infections will generally subside.

There is no definitive therapy that can stimulate bone marrow (myeloid) recovery. Corticosteroids are sometimes used to treat shock induced by overwhelming bacterial infection. However, these drugs are not recommended for the treatment of acute agranulocytopenia because they may mask other bacterial infections.

People with abnormally low levels of immune factors in their blood (hypogammaglobulinemia) associated with acquired agranulocytosis are usually treated with infusions of gamma globulin.

Mouth and throat ulcers associated with acquired agranulocytosis can be soothed with gargles of salt (saline) or hydrogen peroxide solutions. Anesthetic lozenges may also help to relieve irritation in the mouth and throat. Mouthwashes that contain the antifungal drug nystatin can be used to treat oral fungal infection (i.e., thrush or candida). A semi-solid or liquid diet may become necessary during episodes of acute oral and gastrointestinal inflammation. (For more information on this disorder, choose "Candidiasis" as your search term in the Rare Disease Database.)

People with chronic granulocytopenia associated with acquired agranulocytosis need to be hospitalized during acute episodes of infection. These affected individuals should be taught to recognize the early symptoms and signs of acute infection and to seek immediate medical attention when necessary. The therapy for chronically affected individuals is similar to that for the acute form of the disease. People with chronic granulocytopenia, who take low-dose oral antibiotics on a rotating basis, must also be monitored for the infections caused by drug-resistant bacteria as well as infections with opportunistic organisms (e.g., fungi, cytomegalovirus). (For more information on this disorder, choose "Opportunistic Infections" as your search term in the Rare Disease Database.)

What are the risk factors for granulocytopenia primary?

Acquired Agranulocytosis is a rare blood disorder that affects males and females in equal numbers. People who are taking certain medications such as cancer drugs, alkylating agents, anti-thyroid drugs, dibenzepin compounds, or other drugs can be at risk for this disorder.

Is there a cure/medications for granulocytopenia primary?

Granulocytopenia primary, also known as agranulocytosis, is an immunological disorder, in which granulocyte count in the blood reduces below the normal range. Granulocytes are a family of immune cells known as white blood cells (WBCs).

1. When a person is diagnosed with the disease, any suspected medications or offending agents must be stopped.
2. In immunocompromised persons, general care, such as oral hygiene, to control infections is important. Skin infections and abrasions are to be treated immediately and promptly.
3. Immune cells are products of a series of processes that occur in the bone marrow. These processes are initiated by various hematopoietic growth factors. Such factors are prescribed to accelerate production. They include Filgrastim, a granulocyte colony-stimulating factor (G-CSF), Sargramostim, a granulocyte-macrophage colony-stimulating factor, and Pegfilgrastim, a long-acting filgrastim.
4. In case of infections, antibiotics are the choice of treatment as follows:
-Third-generation cephalosporins or equivalents
-Cefepime, carbapenems (e.g., meropenem, imipenem-cilastatin), or piperacillin-tazobactam
-Vancomycin or linezolid in case of methicillin-resistant Staphylococcus aureus.
-Linezolid or daptomycin in cases of vancomycin-resistant enterococcus
-Carbapenems against extended-spectrum beta-lactamase (ESBL) producing gram-negative bacteria.
-Antifungal agents include amphotericin B (preferably lipid formulation), a broad-spectrum azole (e.g., voriconazole), or an echinocandin (e.g., caspofungin).

The initial symptoms are often malaise, fever, and chills, or infections, which usually present in the form of ulcers, necrotizing lesions of the gingiva, the floor of the mouth, buccal mucosa, pharynx, or other sites within the oral cavity,Pharyngitis, with difficulty in swallowing and multiple skin abscesses, could be another presentation of the disease
Neutropenia (lower-than-normal levels of white blood cells and are very vulnerable to infection),Immunodeficiency
Hematopoietic growth factors include injections like Filgrastim, Sargramostim, and Pegfilgrastim,Third-generation cephalosporins or equivalents
Cefepime, carbapenems (e.g., meropenem, imipenem-cilastatin), or piperacillin-tazobactam, Vancomycin, linezolid or daptomycin,Carbapenems
Antifungal agents include amphotericin B (preferably lipid formulation), a broad-spectrum azole (e.g., voriconazole), or an echinocandin (e.g., caspofungin)

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