Clinical Reference
Blood Disorder Information
Comprehensive clinical information authored by Dr. Suparno Chakrabarti and Dr. Mahak Agarwal.
Hodgkin's Lymphoma
Pediatric BMT — Dr. Mahak Agarwal
Hodgkin's Lymphoma in Children
Hodgkin's lymphoma is a distinct cancer of lymphoid tissue derived from germinal B cells of lymph glands, defined by the presence of very characteristic cells called REED-STERNBERG cells or its variants with characteristic immunophenotype in a background of different types of cells.
Types of Hodgkin's Lymphoma
- Nodular sclerosing HL
- Mixed-cellularity subtype
- Lymphocyte-rich or Lymphocytic predominance
- Lymphocyte depleted
- Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL)
What Causes Hodgkin's Lymphoma?
The exact cause of Hodgkin disease is not known. Most Hodgkin's lymphomas occur when an infection-fighting cell called a B cell develops a mutation in its DNA. The mutation tells the cells to divide rapidly and to continue living when a healthy cell would die.
Risk Factors
- Age: Generally Age group 15-35 and 55 years and above are diagnosed with Hodgkin's lymphoma.
- Sex: Mostly Male are suspected to be on higher risk of Hodgkin's lymphoma.
- Previously exposed to the Epstein-Barr virus (EBV), a common virus that causes glandular fever.
- Medical condition that weaken immune system, such as HIV.
- Previous treatment with chemotherapy or radiotherapy for Non-Hodgkin's Lymphoma.
- Painless swelling of the lymph nodes in neck, armpits, groin (swollen glands)
- Fatigue
- Fever and chills that come and go (38°C / 100.4 F)
- Unexplained Itching all over the body
- Loss of appetite
- Soaking night sweats
- Unexplained Weight loss
Other symptoms that may occur
- Coughing, chest pains, or breathing problems if there are swollen lymph nodes in the chest
- Excessive sweating
- Pain or feeling of fullness below the ribs due to swollen spleen or liver
- Pain in lymph nodes after drinking alcohol
- Skin blushing or flushing
Lymph Node Biopsy
Hodgkin's lymphoma is diagnosed from Biopsy of lymph gland or other involved tissues. Histopathology reveals five subtypes: Nodular Sclerosis and Mixed cellularity are more common. Detection of cell surface antigens by Immunohistochemistry (IHC) is confirmatory. Expression of CD30 and CD15 are seen in the RS cells, except in NLPHL, where CD20 is expressed.
Blood tests; Imaging tests (X-rays, CT scan, PET); Bone Marrow Biopsy; Additional Tests: Serum LDH, ECG, Echocardiogram and Lung Function Test before starting treatment.
Staging
- Stage I: Hodgkin disease is found in only 1 lymph node area or lymphoid organ such as the thymus. The cancer has not spread to other areas of the body.
- Stage II: Hodgkin disease is found in 2 or more lymph node areas on the same side of (above or below) the diaphragm.
- Stage III: Hodgkin disease is found in lymph node areas on both sides of (above and below) the diaphragm. It may also have spread to an area or organ near the lymph node areas or to the spleen.
- Stage IV: Hodgkin disease has spread widely through 1 or more organs outside of the lymph system.
Treatment Options
- Chemotherapy
- Radiation therapy
- High-dose chemotherapy and Bone Marrow transplant
Favourable, Limited Stage Disease: Treated with short course chemotherapy and involved field radiotherapy; >95% cure rate.
Unfavourable, Limited Stage Disease: Similar treatment but more intensive; > 90% cure rate.
Advanced Disease: ABVD/BEACOPP >60%-80% cure rates.
However, use of radiotherapy is not favoured in small children unless absolutely necessary.
BMT for Hodgkin's Lymphoma
About 30% patients with advanced disease and 10% patients with Limited disease relapse. Once Hodgkin's lymphoma relapses, the treatment is well-defined:
- Salvage Chemotherapy
- Autologous Peripheral Blood Stem Cell Collection
- High Dose Chemotherapy and Autologous BMT
If the patient is PET negative before BMT, 80% of the patients are cured. If the patient is PET positive, the cure rate is only 20%.
If a patient achieves a complete remission, even in advanced disease, there is no need to perform a BMT upfront.
When is Allogeneic BMT needed?
- When the disease recurs after Autologous BMT
- When PBSC cannot be mobilised for Autologous BMT
There is a strong Graft versus Tumour effect against HL in the setting of Allogeneic BMT. In the setting of High Risk and Relapsed Hodgkin's lymphoma, best results are obtained with HAPLOIDENTICAL DONOR and REDUCED INTENSITY CONDITIONING. 70% patients are cured with this procedure.
Adult BMT — Dr. Suparno Chakrabarti
Hodgkin's Lymphoma in Adults
Hodgkin's lymphoma is a distinct cancer of lymphoid tissue derived from germinal B cells of lymph glands, defined by the presence of very characteristic cells called REED-STERNBERG cells or its variants with characteristic immunophenotype in a background of different types of cells.
Types: Nodular sclerosing HL; Mixed-cellularity subtype; Lymphocyte-rich or Lymphocytic predominance; Lymphocyte depleted; Nodular lymphocyte predominant Hodgkin's lymphoma (NLPHL).
The exact cause of Hodgkin disease is not known. Most Hodgkin's lymphomas occur when an infection-fighting cell called a B cell develops a mutation in its DNA. The mutation tells the cell to divide rapidly and to continue living when a healthy cell would die.
Symptoms: Painless swelling of lymph nodes in neck, armpits, groin; Fatigue; Fever and chills (38°C/100.4F); Unexplained Itching; Loss of appetite; Soaking night sweats; Unexplained Weight loss; Coughing/chest pains/breathing problems; Excessive sweating; Pain or fullness below ribs; Pain in lymph nodes after drinking alcohol; Skin blushing or flushing.
Diagnosis by lymph node biopsy; Histopathology reveals subtypes; IHC is confirmatory (CD30 and CD15 in RS cells, CD20 in NLPHL); Blood tests; Imaging (X-rays, CT, PET); Bone Marrow Biopsy; Additional tests: Serum LDH, ECG, Echocardiogram, Lung Function Test.
Staging: I–IV
Stage I: 1 lymph node area; Stage II: 2+ areas same side of diaphragm; Stage III: both sides; Stage IV: spread widely to organs outside lymph system.
Treatment
Favourable Limited: >95% cure rate. Unfavourable Limited: >90% cure rate. Advanced: ABVD/BEACOPP 60–80%. BMT for relapsed cases: Salvage Chemo → Autologous PBSC collection → High Dose Chemo + Autologous BMT.
PET negative before BMT: 80% cured. PET positive: 20% cure rate. Allogeneic BMT when disease recurs after Autologous BMT or PBSC cannot be mobilised. Haploidentical Donor with Reduced Intensity Conditioning: 70% cure rate.