Case 20 - Haematologcal Malignancies Flashcards
What is normal lymph node anatomy?
200-400 lymph nodes in the neck
Act as filters for the lymphatic fluid
Fluid leaks out of capillaries > interstitial fluid > lymphatic system > jugular vein > SVC
Have a hilum, and an outer cortex (B cells), inner germinal centre is where there is proliferation of lymphocytes
When the lymphocytes in the cortex proliferate upon recognition of a pathogen, it increases the size of the lymph node and causes lymphadenopathy
What is defined at lymphadenopathy in adults and children?
Most children have lymphadenopathy at some point
>2cm width in a lymph node in a child is lymphadenopathy
>1cm is adult lymphadenopathy
But any persistent Sx associated with a swollen node is relevant
How will inflammatory lymphadenopathy present?
The lymph node will be hot, and swollen, fluctuating in size
Often tender too
Associated illness or symptoms often alongside the lymphadenopathy
Could be a head or neck illness
Could be cervical TB (in immunocompromised) - becomes very red around the area and may burst through the skin and seep pus
Why might lymph nodes be malignant?
Could due to metastasis do the lymph nodes e.g. in cervical lymph nodes could be due to head, neck, throat, thyroid cancer etc.
These are fixed and irregular
Could also by lymphoma - cancer of the lymphocytes themselves
How do you test for inflammatory lymph nodes?
Based on history of infection
Can do BTs e.g. WCC and CRP to check for inflammation
CXR for TB or to look at hilar lymph nodes
What is malignant lymphoma?
A cancer of the lymphocytes - does not have to be based at the lymph nodes, can be anywhere that lymphocytes are found - usually non-Hodgkins
More common in B cells than T cells
What are RFs for malignant lymphoma?
FHx EBV HPV Smoker Alcohol Caucasian
But most have unknown cause
What are symptoms of lymphoma?
Non-specific symptoms, like tiredness, lethargy Painless, slow growing lump May have pain Dysphagia Odynophagia
B symptoms are constitutional:
- Night sweats
- Weight loss
- Fever
Assessment of lymphoma?
Will do full history
Reticuloendothelial exam
Head and neck exam including fibreoptic endoscopy in the ENT head and neck lump clinic
Then can either take a USS guided FNA, or do a biopsy
Biopsy is more invasive but gives a more definite diagnosis
USS
CT/MRI
PET
Histology of lymphoma?
In Hodgkins lymphoma, will see the presence of Reed-Sternberg cells ‘owl eyes’ appearance
In non-Hodgkins will just see abnormal cells in an increased number depending on the type of non-Hodgkins
Staging of lymphoma
Using Ann Arbor system:
- Stage 1 = only one group of nodes
- Stage 2 = 2+ groups of nodes but only on one side of the diaphragm
- Stage 3 = on both sides of the diaphragm
- Stage 4 = spread to other organs
What are the types and subtypes of lymphoma?
Hodgkins Non-Hodgkins - high or low grade -Diffuse B cell -T cell -Follicular
What is Hodgkin’s lymphoma?
Presence of Reed-Sternberg cells
Peaks between 15-30y and 50y+
Relapse rare after 5y is rare
Can be:
-Lymphocyte predominant
-Nodular sclerosis - collagen bound nests of Hodgkins –Mixed cellularity
-Lymphocyte depleted
Will see inflammatory cell infiltration too, this is not seen in non-Hodgkins lymphoma
What are the types of non-hodgkins lymphoma?
Diffuse B cell 60y median age Most common type Aggressive but responds well to treatment (high grade) LDH is associated with prognosis
T cell
Can be of the skin and difficult to diagnose
High grade
Follicular
Median age is 60y
Chemo/radiotherapy if there’s symptoms
Seen as incurable due to relapse, but controllable - low grade
Treatment of Hodgkin’s lymphoma?
Assess the stage of Hodgkins lymphoma
1, 2, 3A can be treated with radiotherapy alone
3B and 4 treated with ABVD chemotherapy
Treatment of non-Hodgkin’s lymphoma?
May not treat asymptomatic, low grade, but can use radiotherapy
But use chemo for high grade - RCHOP
What is leukaemia?
Cancer of the blood cells
If it is of the blood cell precursor, it is called acute _-blastic
The immature cells crowd the bone marrow so it is unable to make healthy RBC
In chronic, increased numbers in the blood film
Can be myeloid line - i.e derivatives of the myeloid line - baso/eosino/neutrophil
Or of the lymphocytic line of cells
What are the types of leukaemia.
Chronic myelocytic leukaemia
Acute myeloblastic leukaemia
Chronic lymphocytic leukaemia
Acute lymphoblastic leukaemia
What is acute lymphoblastic leukaemia?
Uncontrolled proliferation of B and T cells Most common Ca in childhood Tranfusions Fluids Chemotherapy Marrow transplants High cure rate in children
What is acute myeloid leukaemia?
Uncontrolled proliferation of the myeloid line
Most common leukaemia in adults
Transfusions
Fluids
Bone marrow transplant
Considered curable, but quick death if untreated
What is chronic myeloid leukaemia?
Uncontrolled clonal proliferation of myeloid cells
Treatment is genotype specific
Stem cell transplant
Variable survival dependent on the phase of the disease
What is chronic lymphocytic leukaemia?
Most common leukaemia Malignant clone of functionally incompetant B cells Drugs if Sx Radiotherapy Stem cell transplant Variable prognosis
Differentials for leukaemia?
Hameatological malignancies
Thrombotic thrombocytopenic purpura
Meningococcal sepsis
Haemolytic uraemic syndrome
Symptoms of leukaemia?
Often non-specific Purpuric rash Fatigue Increased bruising Bleeding gums
What is myeloma?
Cancer of the plasma cells - form deposits in bone and cause osteolytic lesions on X-ray
This leads to decreased erythropoeisis and decreased production of platelets = anaemia and thrombocytopenia
The cells proliferate and produce excess proteins - known as para-proteins
Bence Jones proteins are those found in the urine
Presnts with non-specific symptoms but prone to bone pain but present with hypercalcaemia
Epidemiology of Myeloma?
Often in older people
Afro-carribean
More prevalent in those with MGUS - monoclonal gammopathy of undetermined significance - where there are elevated free light chains are in the blood and have a higher risk of progressing on to myeloma
Tests for myeloma?
FBC - normochromic, normocytic anaemia
High ESR, calcium, urea and cretainine
Bone marrow biopsy
Urine electrophoresis for Bence Jones proteins
Diagnosis is by serum electrophoresis and a monoclonal protein band
X-ray for lytic lesions
Supportive treatment for myeloma?
Analgesia
Bisphosphonates
Correct anaemia with EPO
Fluids
Chemotherapy for myeloma?
Can do chemotherapy
Can consider stem cell transplant when protein levels are reduced
Symptoms of myeloma
Backache Sx of anaemia Recurrent infections Bleeding Sx of high calcium: -Weight loss -Anorexia -Nausea -Polydipsia -Polyuria
