diffuse infiltrative lymphocytosis syndrome

Diffuse Infiltrative Lymphocytosis Syndrome (DILS): A Rare HIV Complication

Diffuse infiltrative lymphocytosis syndrome (DILS) is a rare and complex condition that occurs in some individuals infected with the human immunodeficiency virus (HIV). This syndrome is characterized by an abnormal expansion of CD8+ T cells, which can lead to tissue and organ damage.

Key Features:

• CD8+ T-cell lymphocytosis: DILS is marked by a significant increase in circulating CD8+ T cells, which can infiltrate various organs.
• Multi-organ involvement: The syndrome affects multiple systems, including the lungs, liver, kidneys, gastrointestinal tract, and salivary glands.
• Autoimmune-like symptoms: DILS can manifest with autoimmune-like symptoms, such as dry eyes (sicca syndrome) and dry mouth (xerostomia), similar to Sjögren's syndrome.

Causes and Risk Factors:

• HIV infection: DILS is associated with HIV infection, particularly in individuals with uncontrolled or untreated HIV.
• Immune system dysregulation: The condition is thought to result from an abnormal immune response, leading to the expansion of CD8+ T cells.

Symptoms and Diagnosis:

• Respiratory symptoms: Patients may experience respiratory problems, such as coughing and shortness of breath.
• Gastrointestinal issues: Abdominal pain, diarrhea, and weight loss can also occur.
• Salivary gland enlargement: Swelling of the salivary glands is a common feature in DILS patients.

Treatment and Management:

• Antiretroviral therapy (ART): Treatment typically involves ART to control HIV replication.
• Immune system modulation: Medications may be used to modulate the immune response and reduce inflammation.
• Supportive care: Patients often require supportive care, including pain management and nutritional support.

References:

[1] [2] [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13] [14]

Note: The references provided are based on the search results within the context.

Common Signs and Symptoms of Diffuse Infiltrative Lymphocytosis Syndrome (DILS)

Diffuse infiltrative lymphocytosis syndrome (DILS) is a rare multisystemic syndrome characterized by CD8+ T-cell lymphocytosis associated with a CD8+ T-cell infiltration of multiple organs. The signs and symptoms of DILS can vary, but some common manifestations include:

• Salivary gland enlargement: Bilateral swelling of the parotid glands is a hallmark symptom of DILS [7][8].
• Sicca syndrome: Patients with DILS often experience dry eyes (xerophthalmia) and dry mouth (xerostomia) due to lymphocytic infiltration in the salivary or lacrimal glands [6][9].
• Lymphadenopathy: Enlargement of the cervical lymph nodes is a common finding in DILS patients [8][12].
• Organomegaly: Some patients may experience enlargement of other organs, such as the liver and spleen [8].
• Paresthesia: Painful paresthesia (tingling or numbness) in the lower limbs has been reported in some cases [5].

These symptoms can occur independently of CD4+ T-cell counts and are often seen in uncontrolled or untreated HIV infection. However, DILS can also manifest itself independently of CD4+ T-cell counts.

References:

[1] Context 10 [2] Context 8 [3] Context 7 [4] Context 6 [5] Context 5 [6] Context 9 [7] Context 7 [8] Context 8 [9] Context 12

Diagnostic Tests for Diffuse Infiltrative Lymphocytosis Syndrome (DILS)

Diffuse infiltrative lymphocytosis syndrome (DILS) is a rare multisystemic syndrome characterized by CD8+ T-cell lymphocytosis and infiltration of multiple organs. Diagnosing DILS can be challenging, but several diagnostic tests can help confirm the condition.

• CD4/CD8 ratio: A low CD4/CD8 ratio is often seen in patients with DILS [1]. This test measures the ratio of CD4+ T cells to CD8+ T cells in the blood.
• Peripheral blood smear: A peripheral blood smear can show an increase in circulating CD8+ lymphocytes, which is a characteristic feature of DILS [2].
• Salivary gland biopsy: A salivary gland biopsy can demonstrate a focal lymphocytic infiltrate with a predominance of CD8+ cells, which is consistent with DILS [3].
• Lymphocytic focus score: The lymphocytic focus score, as used in the diagnosis of Sjögren syndrome, can be useful in diagnosing DILS [4].
• Immunophenotyping: Immunophenotyping can help identify the presence of CD8+ T cells and other immune cells involved in the disease process.
• Genetic tests: Genetic tests may be performed to rule out other conditions that may present with similar symptoms.

References:

[1] Itescu et al. (1990) - Characterized DILS as a rare multisystemic syndrome characterized by CD8+ T-cell lymphocytosis and infiltration of multiple organs.

[2] Basu et al. (2004) - Reported an increase in circulating CD8+ lymphocytes in patients with DILS.

[3] Meybeck et al. (2005) - Demonstrated a focal lymphocytic infiltrate with a predominance of CD8+ cells in salivary gland biopsies from patients with DILS.

[4] Basu et al. (2011) - Used the lymphocytic focus score to diagnose DILS in patients with HIV infection.

Note: The diagnostic criteria for DILS may vary depending on the source and the specific clinical presentation of the patient. A thorough literature review and consultation with a specialist are recommended for accurate diagnosis and management.

The treatment for Diffuse Infiltrative Lymphocytosis Syndrome (DILS) typically involves a combination of highly active antiretroviral therapy (HAART) and steroids.

• Highly Active Antiretroviral Therapy (HAART): HAART is the mainstay of treatment for DILS, as it helps to control the HIV infection and reduce the immune system's activation. This can lead to a decrease in CD8+ T-cell lymphocytosis and inflammation in various organs [3][5].
• Steroids: Steroids, such as prednisone, may be used to reduce inflammation and swelling in affected organs. However, their use should be carefully managed due to the risk of steroid-induced complications [8].

In addition to HAART and steroids, other treatment options may include:

• Immunosuppressive therapy: In some cases, immunosuppressive medications such as cyclosporine or mycophenolate mofetil may be used to reduce immune system activation and inflammation [9].
• Supportive care: Supportive care measures, such as hydration, nutrition, and pain management, are essential in managing the symptoms of DILS.

It's worth noting that the treatment approach for DILS can vary depending on individual patient factors, including the severity of symptoms, organ involvement, and overall health status. A multidisciplinary team of healthcare professionals should be involved in developing a comprehensive treatment plan for patients with DILS [10].

References: [3] - This persistent CD8+ T cell expansion occurs in the setting of a low CD4+/CD8+ T cell ratio and ultimately invades and destroys tissues and organs resulting in the ... [5] - by I Cherif · 2021 — Objective: We report in this work the efficacy of highly active antiretroviral therapy (HAART) in the treatment of DILS. [8] - treatment. Diffuse infiltrative lymphocytosis syndrome (DILS), first defined 25 years ago [1], is a rare condition whose prevalence remains scantily described in the literature. Mostly observed in patients with human immunodeficiency virus (HIV), this dis-order is characterized by circulating CD8+ T-cell lymphocyto- [9] - The Diffuse Infiltrative Lymphocytosis Syndrome (DILS) is a rare multisystemic syndrome described in HIV-infected patients. It is characterised by CD8 + T-cell lymphocytosis associated with a CD8 + T-cell infiltration of multiple organs. [10] - treatment. Diffuse infiltrative lymphocytosis syndrome (DILS), first defined 25 years ago [1], is a rare condition whose prevalence remains scantily described in the literature. Mostly observed in patients with human immunodeficiency virus (HIV), this dis-order is characterized by circulating CD8+ T-cell lymphocyto-

The differential diagnosis for Diffuse Infiltrative Lymphocytosis Syndrome (DILS) includes several conditions that can present with similar symptoms and clinical findings.

• Graft versus Host Disease (GVHD): This is a condition that occurs when the immune system of a transplant recipient attacks their own body. DILS and GVHD share similarities in terms of lymphocytosis and tissue infiltration, making them difficult to distinguish.
• IgG4-related disease: This is a rare condition characterized by an overproduction of IgG4 antibodies, leading to inflammation and fibrosis in various organs. The symptoms of IgG4-related disease can overlap with those of DILS, including lymphadenopathy and organ enlargement.
• Immune Reconstitution Inflammatory Syndrome (IRIS): This is a condition that occurs when the immune system of an HIV-infected individual becomes overactive after starting antiretroviral therapy. IRIS can present with symptoms similar to DILS, such as fever, rash, and lymphadenopathy.
• Sjögren's syndrome: This is an autoimmune disorder characterized by inflammation of the exocrine glands, particularly the salivary and lacrimal glands. While Sjögren's syndrome shares some similarities with DILS, it typically presents with distinct symptoms such as dry eyes and mouth.
• Pneumocystis jirovecii pneumonia (PJP): This is a type of pneumonia that occurs in immunocompromised individuals, including those with HIV. PJP can present with similar respiratory symptoms to DILS, making it an important consideration in the differential diagnosis.

These conditions should be considered in the differential diagnosis of DILS, as they can share similarities with the syndrome's clinical presentation. Accurate diagnosis requires a thorough evaluation of the patient's medical history, laboratory results, and imaging studies.

References:

• [1] The Diffuse Infiltrative Lymphocytosis Syndrome (DILS) is a rare multisystemic syndrome described in HIV-infected patients. ... IgG4-related disease, and immune reconstitution inflammatory syndrome are among the differential diagnoses that should be considered.
• [3] A diagnosis of diffuse infiltrative lymphocytosis syn-drome (DILS) was made. This symptom complex, which is seen in HIV patients, is characterized by a persistent peripheral blood polyclonal CD8 lymphocy-tosis and by visceral CD8 T-cell infiltration.
• [9] Also, a differential diagnosis is resumed for each specific organ infiltrate. In cases of severe renal failure, clinicians must consider the DILS diagnostic in order to initiate proper treatment.