Lymphoma

Lymphoid neoplasms

General presentation

• Lymphadenopathy - nontender, firm, rubbery
  • Neck most common
• Constitutional symptoms - fever, night sweats, weight loss
• Fatigue
• Pruritis
• Masses on CXR
• Hepatomegaly
• Splenomegaly
• Cytopaenias
• Hypercalcaemia, high LDH, high uric acid

Hodgkin Lymphoma

• Epidemiology
  • Usually affects people 20-40yo, and adults >50yo
• Pathophysiology
  • Characterised by the presence of Reed-Sternberg cells on histology
  • Subtypes
    • Classic HL (90%)
      • Nodular sclerosis cHL
      • Mixed cellularity cHL
      • Lymphocyte-rich cHL
      • Lymphocyte depleted cHL
    • Nodular lymphocyte predominant HL
• Presentation
  • Mostly asymptomatic lymphadenopathy involving cervical nodes
  • Sometimes constitutional B symptoms and pruritis
• Staging
  • CT and FDG-PET
  • Lugano classification (derived from Ann Arbor staging with Cotswolds modifications)

Stage I — Involvement of a single lymph node region (eg, cervical, axillary, inguinal, mediastinal) or lymphoid structure such as the spleen, thymus, or Waldeyer's ring.

Stage II — Involvement of 2 or more lymph node regions or lymph node structures on the same side of the diaphragm. Hilar nodes should be considered to be "lateralized" and when involved on both sides, constitute stage II disease. For the purpose of defining the number of anatomic regions, all nodal disease within the mediastinum is considered to be a single lymph node region, and hilar involvement constitutes an additional site of involvement. The number of anatomic regions should be indicated by a subscript (eg, II-3).

Stage III — Involvement of lymph node regions or lymphoid structures on both sides of the diaphragm. This may be subdivided stage III-1 or III-2: stage III-1 is used for patients with involvement of the spleen or splenic hilar, celiac, or portal nodes; and stage III-2 is used for patients with involvement of the paraaortic, iliac, inguinal, or mesenteric nodes.

Stage IV — Diffuse or disseminated involvement of 1 or more extranodal organs or tissue beyond that designated "E," with or without associated lymph node involvement.

All cases are subclassified to indicate the absence (A) or presence (B) of the systemic symptoms of significant unexplained fever, night sweats, or unexplained weight loss exceeding 10% of body weight during the 6 months prior to diagnosis.

The designation "E" refers to extranodal contiguous extension (ie, proximal or contiguous extranodal disease) that can be encompassed within an irradiation field appropriate for nodal disease of the same anatomic extent. More extensive extranodal disease is designated stage IV.

Bulky disease: A single nodal mass, in contrast to multiple smaller nodes, of 10 cm or ≥⅓ of the transthoracic diameter at any level of thoracic vertebrae as determined by CT; record the longest measurement by CT scan. The term "X" (used in the Ann Arbor staging system) is no longer necessary.

The subscript "RS" is used to designate the stage at the time of relapse.

• Management
  • Early-stage - CRT
  • Advanced - chemotherapy +/- RTx
  • Splenectomy for symptomatic splenomegaly - rarely indicated
• Prognosis

One point is given for each of the characteristics below present in the patient, for a total score ranging from zero to seven
Serum albumin <4 g/dL
Hemoglobin <10.5 g/dL
Male gender
Age >45 years
Stage IV disease
White blood cell count ≥15,000/microL
Absolute lymphocyte count <600/microL and/or <8 percent of the total white blood cell count
When applied to an initial group of 5141 patients with advanced Hodgkin lymphoma treated prior to 1992 with combination chemotherapy with or without radiation therapy, five-year overall survival (OS) and freedom from progression (FFP) rates according to score were as follows[1]:
Score	Five-year FFP, percent	Five-year OS, percent
0	84	89
1	77	90
2	67	81
3	60	78
4	51	61
5 or more	42	56
When applied to 740 patients with advanced Hodgkin lymphoma treated with curative intent with doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) from 1980 to 2010, five-year OS and FFP rates according to score were as follows[2]:
Score	Five-year FFP, percent	Five-year OS, percent
0	88	98
1	84	97
2	80	91
3	74	88
4	67	85
5 or more	62	67

Non-Hodgkin Lymphoma

• Pathophysiology
  • A group of malignant neoplasms derived from progenerates of B cells and T cells, mature B cells, and mature T cells
  • Subtypes
    • Splenic marginal zone lymphoma (previously splenic lymphoma)
      • Present with splenomegaly, lymphocytosis with anaemia, and thrombocytopaenia
      • Diagnosed on histology from splenectomy or bone marrow biopsy
      • PET/CT can help to diagnose
      • Survival significantly improved after splenectomy
• Staging
  • CT and FDG-PET, followed by biopsy of avid lymph nodes
  • Ideally biopsy obtained prior to initiation of steroids - steroids would lyse lymphoid tissue and may obscure the diagnosis
• Complications
  • Splenomegaly
    • Common occurrence
    • Splenectomy if symptomatic, or in presence of any cytopaenia