Lymphomas

Notes
  • Lymphomas are diverse tumors that arise from reticuloendothelial and lymphatic systems.
  • They are tumors of lymphocytes that result from clonal proliferation of B cells (majority), T-cells or NK cells at various stages of differentiation and/or activation.
  • They are characterized by accumulation of tumor cells in lymph nodes (mainly), spleen, liver, and bone marrow.
  • The four main lymphomas are:

♦ Hodgkin Lymphoma

♦ Non-Hodgkin Lymphomas

♦ Burkitt Lymphoma and

♦ Mycosis Fungoides

  • Classical Hodgkin Lymphomas (CHLs) is characterized by the presence of mononucleated Hodgkin cells and bi- or multinucleated Reed-Sternberg cells all derived from germinal centre B cells.
  • Non-Hodgkin Lymphomas (NHLs) are derived from B lymphocytes, T lymphocytes and uncommonly natural killer cells (NK). They are therefore highly heterogenous (with more than 30 sub-types).
  • NHLs are more common than CHLs.
  • Both NHLs and CHLs are more common in males than in females.
  • CHLs affect teenagers and young adolescents mainly while NHLs affects all ages.
  • Predisposing factors to lymphoma include: immunosuppression, TB, EBV, HPV type 6, genetic factors, radiotherapy, chemotherapy and drugs such as phenytoin.
  • Statistics: prevalence of Diffuse large B-cell lymphoma (DLBL) among lymphomas series globally: 30%–60%; male to female ratio of lymphoma in Kenya – 1.64 to 1
Symptoms
  • Painless (though painful on consumption of alcohol) lymphadenopathy that is usually progressive.
  • Weight loss and later cachexia
  • Anaemia
  • Hepatomegally
  • Night sweats
  • Persistent fever
  • Pruritus
  • Splenomegaly
  • Pain with osteolytic lesions and compression fracture when bones are affected.
  • Sepsis (often the main cause of death) due to immune suppression.
  • Symptoms of compression of tissues by lymphomas include: leg edema, jaundice (due to obstruction of bile duct); dyspnea and wheezing (due to tracheobronchial compression); neuralgic pain (due to compression of nerve root); paraplegia (due to compression of spinal cord); laryngeal paralysis and Horner syndrome (reduced pupil size, a drooping eyelid and decreased sweating on the face)
Diagnosis
  • Chest x-ray
  • Lymph node excision biopsy (and a look at the evidence of Reed-Sternberg cells)
  • Fine needle aspirations (FNA) – for screening NOT diagnosis.
  • CT of chest, abdomen, and pelvis
  • Positron emission tomography (PET) for staging.
  • MRI (when neurologic symptoms are noted).
  • For CHLs the following immunochemistry tests are very useful:

Test

Immunochemistry test

NHL Lymphoid markers

CD45 (pan-) CD20 (B-) and CD3 (T-)

CHL

CD15 and CD30

T-cell lymphomas

CD 30

 To exclude B-cell lymphoma the following tests are also done: CD45, CD3 and CD20.

Follicular lymphoma

Bcl2 stain and CD45, CD3 and CD20

Burkitt’s lymphoma

CD45, CD3 and CD20 and

_To exclude ALL TDT test is done.

_To exclude Burkitt’s like lymphoma Ki67 is done

  • Additional tests that are required during the follow-up visits after a treatment regime include the following;

♦ FBC, BUN and Ca

♦Alkaline phosphatase, Creatinine, albumin, LFTs

♦ LDH twice per year for 2 years

♦ TSH in cases where the patient has undergone irradiation

♦ CT scan when relapse is suspected from clinical or laboratory test results.

Differential
  • Allergic reaction
  • Cytomegalovirus Infectious
  • Human immunodeficiency virus (HIV)
  • Mononucleosis
  • Sarcoidosis
  • Serum Sickness
  • Small Cell Lung Cancer
  • Syphilis
  • Toxoplasmosis
  • Tuberculosis
  • Other solid tumors
Prevention
  • Avoid, if possible, exposure to the risk factors.
Management
  • NHL;

♦Since it has heterogenous etiology, it is more complicated to manage than CHL.

♦When DLBL is diagnosed the treatment of choice is CHOP (cyclophosphamide, doxorubicin (hydroxydaunomycin), vincristine (Oncovin®) and prednisolone) 6-8 cycles with addition of Rituximab if the patient is CD 20 positive.

♦Radiotherapy for treatment and palliation.

♦Intrathecal chemotherapy and craniospinal radiation is recommended in cases where CNS is affected

  • CHL;

Limited disease

_Combined administration of of doxorubicin, bleomycin, vinblastine and dacarbazine (ABVD) - 2 cycles. These cycles are  followed by radiation therapy or 2 to 4 additional cycles of ABVD in cases where radiation therapy is not available.

Moderate disease

_Combined administration of of doxorubicin, bleomycin, vinblastine and dacarbazine (ABVD) - 4 cycles. These cycles are  followed by radiation therapy or 2 additional cycles of ABVD in cases where radiation therapy is not available.

Advanced disease

_Combined administration of of doxorubicin, bleomycin, vinblastine and dacarbazine (ABVD) – 6 to 8 cycles.

  • Follow up after treatment:

♦Review visits to be done;

_Every 3 months for 2 yrs, then;

_Every 6 months for 3 yrs, then;

_Annually thereafter.

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