Introduction
Blood is an essential connective tissue comprising plasma and cells, including red blood cells, white blood cells, and platelets. Any defect in the quantity or quality of the components of blood leads to clinical disorders ranging from mild to significant symptoms. It is the decrease in the count of white blood cells (lymphocytes) that help the body to defend against cancer, viruses, and other microbes. Lymphocytopenia can be caused due to many causes, which can be inherited or acquired causes. Healthcare providers treat the condition by treating the cause.
What is Lymphocytopenia?
The condition in which the white blood cells are lacking in the individual's blood is called lymphocytopenia. Lymphocytopenia is also termed lymphopenia. Lymphocytes are the cells that protect from infection. Infections can become life-threatening when an individual's T or B cells are absent. Most of the lymphocytes play an essential role in evoking immune action.
Three types of lymphocytes are:
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T-cells - They help destroy the infected cells along with B cells.
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B-cells - Signalling proteins and antibodies are made that help in identifying the attacking bacterial or viral toxins.
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Natural killer cells - NK cells help by killing the cancer cells.
What Are the Causes of Lymphocytopenia?
Lymphocytopenia can be caused due to
Acquired Causes:
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Infections.
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Autoimmune disorders.
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Blood and bone marrow transplants.
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Poor nutrition.
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Consumption of alcohol.
Inherited causes:
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Ataxia telangiectasia.
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Common variable immunodeficiency.
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Wiskott-Aldrich syndrome.
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Severe combined immunodeficiency syndrome.
What Is Digeorge Syndrome?
DiGeorge syndrome is caused by the absence of a tiny portion of chromosome 22. The missing part is responsible for the underdevelopment of several body systems.
Causes:
The deletion of genes from chromosome 22 can occur randomly in the father's sperm or the mother's egg, or it can occur during the development of the fetus.
It is rare that this mutation is inherited and passed on to a child from their parent with the same genetic picture showing or not presenting with any symptoms.
Symptoms:
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Bluish skin and a heart murmur can occur due to a lack of oxygen-rich blood circulating in the body due to a heart defect.
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Recurrent infections.
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Cleft palate (slit or gap in the palate).
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Difficulty in feeding.
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Failure to gain weight.
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Delayed development.
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Behavior issues.
Complications:
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Heart Defects - DiGeorge syndrome can cause heart defects that result in cyanosis due to an insufficient supply of oxygen-rich blood.
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Hypoparathyroidism - The levels of calcium and phosphorus are regulated by the four parathyroid glands in the neck of the body. DiGeorge syndrome can cause more minor parathyroid glands that secrete less parathyroid hormone (PTH), leading to hypoparathyroidism.
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Thymus Gland Dysfunction - The thymus gland is where T cells mature. Alternation in the functioning of the thymus gland can lead to improper protection from infections.
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Cleft Palate - It is a common condition seen in the DiGeorge syndrome that presents as a gap or slit in the palate or lip.
What Is Wiskott Aldrich Syndrome?
The condition known as Wiskott Aldrich syndrome (WAS) causes immunological deficiencies and a decreased capacity for blood clotting..
Signs and Symptoms of WAS Include:
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Easy bleeding or bruising due to a decreased size and number of platelets.
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Susceptibility to immune disorders, infections, and inflammatory disorders.
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Increased risk for developing some cancers (like lymphoma).
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Eczema (a skin condition) is also common in people with WAS.
Causes of WAS Include:
It is an inherited X-linked disorder caused by genetic changes in the gene named WAS. Primarily it affects males.
WAS-related disorders include the following:
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X-linked neutropenia (XLN).
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X-linked thrombocytopenia (XLT).
They are grouped into one category as mutations in WAS gene cause all the diseases. And show overlapping symptoms, which can be severe to mild. Wiskott-Aldrich syndrome is the most severe among all WAS-related disorders.
What Is Severe Combined Immunodeficiency Syndrome?
Severe combined immunodeficiency syndrome is an immunodeficiency disease in which T lymphocyte and B lymphocyte functions are absent. SCID (severe combined immunodeficiency disease) is fatal without a treatment like corrective gene therapy or stem cell transplant. Nearly 15 different genetic defects are responsible for causing SCID. Due to these genetic defects, the individual is highly susceptible to various infections. This condition is one of the serious forms of primary immunodeficiency disease (PI). Treatments like bone marrow transplant (hematopoietic stem cell transplantation) help treat the condition and offer promising results to the patient.
Causes of SCID:
SCID is an autosomal inherited disease associated with several genetic defects, which include gene mutations encoding CD45, LAT, and coronin 1A. In nearly 10 percent of the individuals suffering from SCID, no identifiable defect in the genes was there to explain the clinical findings.
Clinical Presentation:
The prevalence of SCID is rapidly changing as screening for SCID with the help of T cell receptor excision (TREC) to identify infants with the risk of developing SCID before the occurrence of symptoms. This helps in the intervention of treatment earlier and improves survival. Infants with SCID show no external physical findings that can distinguish them from normal newborns, and they appear to be clinically fit and well until the infection comes. If the newborn is not diagnosed with SCID with the help of TREC screening, symptoms keep appearing and increase in intensity. The infections are not similar to the infections of normal infants, such as regular colds. Infects of an infant with SCID are serious and life-threatening, including severe viral respiratory infections, pneumonia, meningitis, and infections in the blood.
Treatment:
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Infants suffering from this life-threatening condition need love and support that parents can provide.
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They will have to bare hospitalizations repeatedly, which can be associated with painful procedures.
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Parents must be courageous and rely upon all of their inner strength and resources to learn to handle the stress and anxiety of this clinical condition. They must have useful and well-defined support groups and coping mechanisms.
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The parents' demands of energy and time taking care of someone with SCID can be tedious and overwhelming. If the parents have two children suffering, they must remember that love and care should be equally shown to both kids. Parents must spend enough energy and time and maintain their relationship without neglecting each other.
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Family counseling is also advised to keep relationships together.
What Are the Symptoms and Treatment Options of Lymphocytopenia?
The following are the symptoms of lymphocytopenia:
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Cough.
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Pain in the joints.
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Night sweats.
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Weight loss.
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Enlarged lymph nodes.
Treatment Options for Lymphocytopenia:
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Treatment for lymphocytopenia depends on the underlying cause. As immunity is compromised, therapy to prevent infection-related complications can be administered.
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Few mild lymphocytopenias need no treatment, while others need treatment for the cause. Treating the underlying cause successfully helps improve the patient's clinical condition.
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The absence of symptoms is also seen in patients for whom no treatment is recommended.
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Treating and managing medical conditions can prevent certain types of lymphocytopenia.
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The condition can be managed by maintaining a proper lifestyle and healthy diet and avoiding the consumption of alcohol. Preventing lymphocytopenia is inevitable as it is primarily an inherited condition.
Conclusion:
It is observed that lymphocytopenia is a condition that is caused due to acquired as well as inherited conditions. Inherited conditions are mostly occurring through a change in genes. Early diagnosis of the disorder is necessary to obtain better treatment outcomes. Follow-up with the doctor should be maintained as the chance of recurrence is high.
