Lymphoid neoplasms are divided into the two main groups: Hodgkin and Non-Hodgkin Lymphomas. The former ones are the most curable due to the specific pathogenesis of the ailment and recent advance in the methods of treatment (5 years survival for the patients with favorable prognosis 98% and 85% with less benign one) . The malignant cells form in the lymphatic system; mainly they are represented by white blood cells, namely, immature B-lymphocytes. HL may take place in any part of the lymphatic system. First this disease was described by Dr. Thomas Hodgkin in 1832 as a type of cancer of the lymph nodes.
Are you more of a visual learner? Check out our online video lectures and start your hematology course now for free!

Image: “Macroscopic aspect of the cut surface of a human lymph node replaced by lymphoma. The node was about 6 x 4 cm (2.5" x 1.5"). The lymphoma is the nodules of pinkish tissue surrounded by yellowish fatty tissue. Upon histological examination, the node contained follicular lymphoma.” by Emmanuelm. License: CC BY 3.0

Image: “Macroscopic aspect of the cut surface of a human lymph node replaced by lymphoma. The node was about 6 x 4 cm (2.5″ x 1.5″). The lymphoma is the nodules of pinkish tissue surrounded by yellowish fatty tissue. Upon histological examination, the node contained follicular lymphoma.” by Emmanuelm. License: CC BY 3.0


Epidemiology

Hodgkin lymphoma is not very common—it accounts for 2-3 per 100 000 cases in Europe and the USA, this disease occurs most often in North Europe and Canada. Men are more susceptible to this type of tumor growth, apart from nodular sclerosing subtype, which is more spread in young females. Being infected with Epstein-Barr virus increased the probability of contracting HL as well as having a first-line relative suffering from HL. Young age is another predominant factor for the development of HL.

Clinical Presentation

  • Painless lymph nodes(asymptomatic)
  • Significant weight loss
  • Fever of unknown origin.
  • Night sweats.
  • Cough, breathlessness, chest pain.
  • Skin itchiness
  • Pain in the places of malignant growth.
  • Backache and pain in the joints.
  • Palpable with no pain lymph nodes in the cervical area, axilla, or inguinal area.
  • Engagement of Waldeyer ring (back of the throat, including the tonsils), occipital or epitrochlear areas.
  • Splenohepatmegaly.
  • Massive mediastinal lymphadenopathy is responsible for the development of Superior vena cava syndrome.
  • Paraneoplastic syndromes, including cerebellar degeneration, neuropathy, Guillain-Barre syndrome or multifocal leukoencephalopathy may occur due to the involvement of CNS in to the pathogenic process.

Staging

Stage 1: 1 group of nodes or 1 organ is affected with HL.

Stage 2: HL affects 2 groups of nodes or 1 organ and 1 or more groups of nodes, the two sites of process on the same side of the diaphragm.

Stage 3: HL is on the both sides of the diaphragm (multiple organs and groups of nodes are affected).

Stage 4: HL affects many groups of nodes and the liver, bones, lungs.

Diagnosis

  • Histologic diagnosis of Hodgkin lymphoma is compulsory method of diagnostics, which is excisional lymph node biopsy. Reed-Sternberg cells are usually found in the biomaterial taken from the nodes.
  • Bone marrow biopsy is indicated in late stages of the disease
  • Lumbar puncture and magnetic resonance imaging are informative in the cases of CNS involvement

Laboratory studies

  • FBC ( in order to detect anemia, lymphopenia, neutrophilia, or eosinophilia)
  • ECR
  • Test for HIV, hepatitis C and B
  • Lactate dehydrogenase
  • Serum creatinine
  • Alkaline phosphatase

Imaging studies

  • CT scans of the chest, abdomen, and pelvis ( used in order to detect abnormal findings include enlarged lymph nodes, hepatomegaly and/or splenomegaly, lung nodules or infiltrates, and pleural effusions)
  • Positron emission tomography is informative in diagnostics of initial stages of the disease.

Differential Diagnosis

DD is usually emphasized on the conditions followed by enlargement of lymphatic nodes.

  • Cytomegalovirus
  • Infectious Mononucleosis
  • Systemic Lupus Erythematosus
  • Non-Hodgkin Lymphoma
  • Sarcoidosis
  • Small Cell Lung Cancer
  • Serum Sickness
  • Syphilis
  • Toxoplasmosis
  • Tuberculosis

Management

Treatment of HL depends on the initial health condition, age of the patient, type of Hodgkin disease, stage of the disease, result of the FBC.

  • Induction chemotherapy, Radiation therapy, Salvage chemotherapy are directed on the malignant growth determination.
  • Hematopoietic stem cell transplantation

Early stage Hodgkin lymphoma:

  • Chemotherapy (2-4 cycles of ABVD containing Adriamycin (doxorubicin), bleomycin, vinblastine and dacarbazine).
  • Radiotherapy

Advanced stage Hodgkin lymphoma

  • Chemotherapy (ABVD for up to 8 cycles)
  • Other combinations of chemotherapy:
    • ChlvPP – chlorambucil, vinblastine, procarbazine and prednisolone
    • Stanford V – mustine, doxorubicin, vinblastine, vincristine, bleomycin, etoposide and steroids
    • BEACOPP – bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine and prednisolone
    • GemP – gemcitabine, cisplatin and prednisolone
    • ESHAP – etoposide, prednisolone, cytarabine and cisplatin
  • Radiotherapy
  • Steroids

Recurrent lymphoma or unresponsive to the treatment HL

  • Bone marrow / stem cell transplantation
  • BEAM chemotherapy

Review Questions

The correct answers can be found below the references.

1. A 23 years old man from Canada complains about fatigue and recent weight loss, he also, noticed that his neck lymph nodes enlarged, they are not painful on a touch, moderately movable. He also, feels “a frog in the throat” and difficulty with swallowing, which he finds painful. His sibling (sister) 19 years old has recently undergone treatment for Hodgkin`s lymphoma. His FBC revealed anemia. What is the most probable primary diagnosis for the condition described above?

  1. Hodgkin`s lymphoma.
  2. Non-Hodgkin`s lymphoma.
  3. Syphilis.
  4. Sore throat (infectious mononucleosis).
  5. Leukemia.

2. A 20 years old women from the US complains about fever, cough, pain in her chest, and swollen nodes in her neck and underarm. She considers herself ill for the last 2-3 weeks. The biopsy of the most enlarged lymph node in the neck revealed Reed- Sternberg cells. What is the most probable diagnosis?

  1. Syphilis.
  2. Tuberculosis.
  3. Flu
  4. Hodgkin`s lymphoma
  5. Streptococcal pneumonia.

3. What viral infection accompanied by hepatosplenomegalia may become a predisposing factor for the development of Hodgkin’s lymphoma?

  1. Coxacci virus.
  2. Epstein –Barr.
  3. Mononucleosis.
  4. Hepatitis C
  5. HIV
Lecturio Medical Courses

Leave a Reply

Your email address will not be published. Required fields are marked *