Day #215 - Pancytopenia from Lymphoma

Classification of Lymphoma:

A good review article here, from which I have shared with you some of the best figures. If you read this you realize that the classification of lymphoma is extremely complicated and depends on morphologic appearance, genetics and immunophenotype. Lymphomas can arise from B-cells, T-cells or Natural Killer (NK) cells.

It is far easier to conceptualize non-Hodgkin's lymphoma as follows:

Indolent (survival in years, difficult to treat ~ 1/3 of all new diagnoses):

• Follicular lymphoma, Small Lymphocytic Lymphoma (like CLL but primarily nodal disease), Mantle Cell Lymphoma, Marginal Zone Lymphoma

Agressive (survival in months, curable)

• Diffuse Large B Cell Lymphoma (DLBCL), Anaplastic T Cell Lymphoma, Peripheral T Cell Lymphoma

Super Aggressive (survival in weeks, potentially curable)

• Burkitt's Lymphoma, B Cell Lymphoblastic Leukemia, Adult T Cell Leukemia/Lymphoma, T cell lymphoblastic leukemia

We reviewed the Ann Arbor Staging System:

• Stage I - 1 chain
• Stage II - 2 separate chains, same side of diaphragm
• Stage III - Disease on both sides of diaphragm
• Stage IV - Diffuse involvement of extralymphatic organ (i.e. liver, lung, bone marrow, brain)

There are suffixes appendied to this based on:

• B - B symptoms: fever, Night sweats, 10% weight loss
• S - If spleen is involved

Staging is performed by CT scans of thorax/abdomen/pelvis to document nodal disease.

A bone marrow biopsy can be performed to look for involvement.

Lumbar puncture is indicated in:

• Patients with neurological symptoms
• HIV associated lymphoma
• Patients with bone marrow involvement (and aggressive lymphoma)
  
• Highly aggressive lymphoma
• Patients with 2 extranodal sites
  

Prognosis in NHL depends on tumor subtype. For the Aggressive Lymphomas the International Prognostic Index can be used. Bad prognostic features include

• Age >60
• ECOG greater than 2
• LDH abnormal
• Stage III or IV disease
• More than 1 extranodal site of disease
  

Score/5yr Survival (WITH TREATMENT)

• 0-1: Low Risk ~ 75%
• 2: Intermediate Risk ~50%
• 3: Intermediate Risk ~40%
• 4-5: High Risk ~25%
  

Reiterating the point about excisional biopsy this 2008 publication highlights the very important role of the general surgeon in making the diagnosis! Supraclavicular, axillary or cervical nodes are much higher yield than inguinal nodes.

NB: HIV, Hepatitis B, and Hepatitis C testing should be performed in new diagnoses -- particularly in younger patients.