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Understanding Diffuse Histiocytic Lymphoma: A Guide

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Diffuse histiocytic lymphoma (DHL) is a subtype of aggressive non-Hodgkin lymphoma. Read below to know more.

Medically reviewed by

Dr. Rajesh Gulati

Published At November 27, 2023
Reviewed AtJanuary 2, 2024

Introduction

Diffuse histiocytic lymphoma is a fast-growing non-Hodgkin lymphoma that develops from certain white blood cells called histiocytes. These cells normally help fight infections and remove dead or damaged cells from the body. In diffuse histiocytic lymphoma, the histiocytes grow out of control, crowding out healthy cells. The prognosis depends on the stage of diagnosis and how well people respond to treatment. Many patients achieve remission or cure with the right treatment plan, including chemotherapy, immunotherapy medications, radiation therapy, or stem cell transplants. By educating about diffuse histiocytic lymphoma, people can have constructive conversations with their doctor to develop the best path forward based on their unique situation.

What Is Diffuse Histiocytic Lymphoma?

Diffuse histiocytic lymphoma is a type of non-Hodgkin lymphoma. It occurs when the body produces too many histiocytes, a white blood cell in the immune system. These histiocytes, also known as macrophages, accumulate in the lymph nodes and other organs and make them enlarge.

The uncontrolled growth of histiocytes impacts the body's ability to fight infection and can crowd out healthy cells. Diffuse histiocytic lymphoma typically develops slowly over time, and the cause is unknown. It most often affects older adults, usually over the age of 60. Some common symptoms of diffuse histiocytic lymphoma include:

  1. Swollen lymph nodes in the neck, underarms, abdomen, or groin.

  2. Unexplained weight loss.

  3. Loss of appetite.

  4. Fatigue or lack of energy.

  5. Night sweats.

  6. Fever.

The doctor will review the symptoms and medical history to determine if people have diffuse histiocytic lymphoma. They may order blood tests, imaging scans like CT (computed tomography) or PET (positron emission tomography) scans, and a lymph node biopsy. Treatment options include chemotherapy, radiation therapy, immunotherapy, and stem cell transplants. The prognosis depends on factors like the stage and location of the cancer when diagnosed. Diffuse histiocytic lymphoma can be an aggressive disease, so early diagnosis and treatment are critical. See a doctor immediately when experiencing swollen lymph nodes or other symptoms that cause concern.

What Are the Symptoms and Diagnosis of Diffuse Histiocytic Lymphoma?

Diffuse histiocytic lymphoma can cause a variety of symptoms as the disease progresses. A combination of tests is usually required to properly diagnose diffuse histiocytic lymphoma.

Symptoms

  • Swollen lymph nodes of neck, armpit, or groin. The lymph nodes may be painless.

  • Unexplained fever, chills, sweating, or weight loss.

  • Fatigue and decreased energy.

  • Shortness of breath or cough.

  • Abdominal swelling or pain.

  • Anemia (decreased red blood cell count) can cause fatigue, shortness of breath, or pale skin.

Diagnosis

  1. Physical examination and health history. The doctor will check swollen lymph nodes and review the symptoms.

  2. Blood tests to rule out abnormal blood cell counts and lymphoma cells.

  3. Imaging tests like CT scans, PET scans, and MRIs (magnetic resonance imaging) to detect swollen lymph nodes and other signs of lymphoma in the body.

  4. Lymph node biopsy (an enlarged lymph node sample is removed and examined under a microscope to determine if lymphoma cells are present).

  5. Bone marrow biopsy (a sample of bone marrow is taken to rule out if the lymphoma has spread).

An accurate diagnosis of diffuse histiocytic lymphoma can be made with a combination of symptoms, blood tests, imaging, and biopsies. Treatment options depend on the stage and aggressiveness of one’s lymphoma.

What Are the Treatment Options for Diffuse Histiocytic Lymphoma?

Treatment for diffuse histiocytic lymphoma typically involves chemotherapy, targeted therapy, radiation therapy, immunotherapy, or a combination of these approaches. The specific treatment plan will be dependent on factors like the stage and progression of the lymphoma, overall health, and preferences.

  • Chemotherapy: Chemotherapy uses anti-cancer drugs to destroy lymphoma cells. It is often the primary treatment for diffuse histiocytic lymphoma. Multiple rounds of combination chemotherapy, using more than one drug, are usually administered. Common regimens include Cyclophosphamide, Doxorubicin, Vincristine, Prednisone (CHOP), or similar variations. The drugs are given intravenously, targeting dividing cells like lymphoma. Chemotherapy can cause side effects but also often leads to remission.

  • Targeted Therapy: Targeted drug treatments focus on specific vulnerabilities of lymphoma cells. They include monoclonal antibodies like Rituximab that target certain proteins in lymphoma cells. Rituximab is often used in combination with chemotherapy. Other targeted therapies include proteasome inhibitors such as Bortezomib.

  • Radiation Therapy: Radiation uses high-energy beams to kill lymphoma cells. It may be used if lymphoma is localized to a specific body area. Radiation therapy is also sometimes used to relieve symptoms of spreading lymphoma.

  • Immunotherapy: Immunotherapies harness immune system to fight lymphoma. Options include immune checkpoint inhibitors, chimeric antigen receptor (CAR) T-cell therapy, and tumor vaccines. These innovative treatments are still emerging but show promising results for some diffuse histiocytic lymphoma patients.

The prognosis for diffuse histiocytic lymphoma continues to improve with ongoing research into new and improved treatments. By working closely with the oncologist, people can determine an effective course of treatment to overcome this disease.

How Is the Prognosis of Diffuse Histiocytic Lymphoma?

The prognosis for individuals with DHL can vary based on a range of factors, including the patient's overall health, the extent of the disease, response to treatment, and other individual characteristics. Some factors that can influence the prognosis of diffuse histiocytic lymphoma include:

  1. Stage and Spread of Disease: The stage of the disease at diagnosis and whether it has spread to other parts of the body (staging) can significantly impact prognosis. The earlier-stage disease generally has a better prognosis than advanced-stage disease.

  2. Biomarkers and Genetic Characteristics: Specific genetic and molecular characteristics of the lymphoma cells can provide additional information about the behavior of the disease and response to treatment. Some genetic abnormalities may indicate a more aggressive form of the disease.

  3. Response to Treatment: How well the lymphoma responds to initial treatment, such as chemotherapy, can influence prognosis. Achieving complete remission (no evidence of disease) after treatment is associated with a better prognosis.

  4. Age and General Health: Younger and healthier patients tend to tolerate aggressive treatments better and may have a better prognosis. Pre-existing health conditions can also impact a patient's ability to undergo and respond to treatment.

  5. Treatment Options: Advances in lymphoma treatment have improved outcomes for many patients. The availability of targeted therapies, immunotherapies, and stem cell transplantation can impact prognosis.

  6. Time of Diagnosis: Lymphoma subtypes and treatment options can evolve over time as research progresses. Being diagnosed at a time when newer, more effective treatments are available could improve prognosis.

Conclusion

Diffuse histiocytic lymphoma is a complex cancer that requires careful diagnosis and treatment. While the prognosis can vary significantly based on factors like stage and spread, the good news is there are multiple options for managing this disease and extending life. By understanding the nature of DHL, the diagnostic process, and available treatments, people can work closely with their doctor to determine the best course of action based on their unique situation. With vigilance and the latest medical therapies, DHL can be managed. Staying optimistic and maintaining open communication with the healthcare team will serve people well on this journey.

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Dr. Rajesh Gulati
Dr. Rajesh Gulati

Family Physician

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non-hodgkin lymphomalymphoma
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