Friday, 2 May 2014

Lymphoma

Haematological malignancies are varied, and there are many different groupings that all present similarly, but with very different clinical prognoses.
14,000 new diagnoses annually.
A full time GP with 2000 patients would see two patients with previously undiagnosed lymphoma every five years. 
Staged with the Ann Arbor system:




Pathophysiology
Lymphoma is a  heterogeneous group of malignancies resulting from abnormal proliferation within lymphoid tissues, such as lymph nodes, spleen, and bone marrow.
They are split into two groups - non hodgkins and hodgkins lymphoma.

NHL
- about 85% - of patients.
- rate of NHL increases with age.
- May be indolent and slow growing or aggressive.

HL
- Reed-Sternberg cells, which have a typical histological appearance. 
- Bi-modal distribution with peaks in young adults and elderly people

Clinical Features

Predisposed to lymphoma:
Immunodeficiency syndromes, EBV, conditions associated with chronic inflammation including H pylori, AU disesases like Hashimoto's, coeliac or Crohn's.

Lymphadenopathy:
        The most common presentation, and can be the only symptom
        Usually presents as a painless lump, often in the neck or supraclavicular region
        Pain can rarely be precipitated by alcohol intake
        The nodes may fluctuate in size spontaneously.
Constitutional symptoms (referred to as B symptoms, present in about 25%):
        Fever (>38°C)
        Night sweats (drenching)
        Weight loss (unexplained, more than 10% of body weight, in the prior six months).
Pruritis

Abdominal masses, especially in children

Investigations
- Full blood count. This may be completely normal. Other pictures include a normochromic, normocytic anaemia, a pancytopenia, a neutrophilia, or an eosinophilia. Anaemia can reflect, for example, chronic disease or marrow infiltration
- C reactive protein and ESR. May be elevated
- Renal function tests. Required to ensure function is normal prior to treatment
- Liver function. May be abnormal in the absence of liver involvement
- LDH. LDH is normally intracellular but is released as a result of cell turnover and cell breakdown due to pressure effects and stress on surrounding tissue
- Uric acid. Some aggressive NHLs are associated with high urate levels that can precipitate renal failure when treatment is started
- Chest x ray. May reveal lymphadenopathy or a mediastinal mass

Hyperviscosity
Haemotological emergency.  The syndrome can arise when there is an elevation in the blood viscosity due to an increase in either red cells, white cells, or plasma components such as immunoglobulins (Igs). This can occur in conditions such as:
    Waldenstrom's macroglobulinaemia (a sub-type of lymphoma) and myeloma (raised monoclonal Igs)
    Polycythaemia (raised red cell volume)
    Acute leukaemias (elevated leukaemia cells in the peripheral blood)
    Myeloproliferative disorders.

Clinical features vary with the underlying condition but can include lethargy, headaches, visual disturbance, isolated cranial nerve palsies, confusion, decreased conscious level, and myocardial infarction and stroke.



References
http://www.enlightenme.org/learning-zone/you-can%E2%80%99t-ignore-paediatric-back-pain
http://calgaryguide.ucalgary.ca/slide.aspx?slide=Clinical%20Features%20to%20Describe%20Abnormal%20Lymph%20Nodes.jpg
http://calgaryguide.ucalgary.ca/slide.aspx?slide=hodgkin%20lymphoma%20-%20pathogenesis%20and%20clinical%20findings.jpg
http://www.enlightenme.org/learning-zone/epistaxis-child
http://learning.bmj.com/learning/modules/flow/ICH.html?execution=e1s1&locale=en_GB&action=start&sessionTimeoutInMin=90&moduleId=10045108&status=LIVE&_flowId=ICH 
http://www.bmj.com/content/348/bmj.g1721?variant=pdf
http://www.enlightenme.org/learning-zone/mass-not-miss

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