Lymphoma and Hairy Cell Leukaemia - Lymphoproliferative Disorders

Lymphoma

·        Malignant lymphoma = Clonal proliferation of lymphocytes arising in lymph nodes (or other lymphoid tissue). Minor exceptions – can get them in spleen, gut, etc

·        Differentiating lymphoma from leukaemia: was its origin in the bone marrow or lymph nodes?

·        Clinical features:

o  Painless lymphadenopathy: non-tender, rubbery

o  Hepatosplenomegaly

o  Systemic symptoms: fever, nights sweats, weight loss, tiredness

o  Involvement of other areas: skin, CNS, GI, salivary glands 

o  If bone involvement (fairly rare) then preference for bones with red marrow, and may present with bone pain

·        Diagnosis: excision biopsy (not FNA).  Special lab procedures, stains etc.  Warn the lab it‟s coming

·        Classification: 

o  Hodgkin‟s vs non-Hodgkin‟s: histological diagnosis only. No clinical difference. Hodgkin‟s responds better in general. In general, Hodgkin‟s spreads node to node, non-Hodgkin‟s spreads to any node in the body 

o  Low (indolent) vs intermediate vs high (aggressive) grade

o  Staging: Ann Arbor Staging System (Ann Arbor is a place in the USA):

§  1: one lymph node area only

§  2: 2 or more lymph node areas on the same side of diaphragm

§  3: 2 of more lymph node areas on different sides of the diaphragm

§  4: disease in liver, bone marrow or other extra-nodal sites 

§  Symptom status: A = absence of fevers, sweats, weight loss. B = one of unexplained fever > 38.5 C, weight loss > 10 % in preceding 6 months, drenching night sweats [Unusual to include symptom status in cancer staging] 

§  Staging investigations: CT of neck, chest and abdomen.  Bone marrow.  FBC, LFTs, ESR

§  Compared with leukaemia: if its in your bone marrow its everywhere

·        Survival:

o  Hodgkin‟s Disease:

·        Non-Hodgkin‟s Lymphoma:

·        Treatment:

o  Radiology if localised (main side effect is tiredness)

o  Chemo if disseminated

o  Possible bone marrow transplant if chemo fails (permits more toxic dose of chemo)

o  If treatment fails then gradual progression

Hairy Cell Leukaemia

·        Indolent B cell neoplasm.

·        Males to female = 5:1.  Median onset age 50

·        Splenomegaly

·        Wispy changes to cytoplasm of B cell 

·        Purine analogues ® 80% remission