Case 20 - Haematological Malignancies Flashcards
What is bilateral hilar lymphadenopathy on a chest X ray?
Bilateral enlargement of the lymph nodes of the pulmonary hila
What are the differentials for bilateral hilar lymphadenopathy?
Sarcoidosis Tuberculosis Lymphoma Glandular fever Carcinoma Secondary metastases Lung cancer
What is a lymphoma?
Malignant proliferations of the lymphocytes which accumulate in the lymph nodes causing lymphadenopathy
Which organs can lymphomas infiltrate?
Any which are part of the reticuloendothelial system:
- spleen
- liver
- bone marrow
What are the two histological types of lymphoma?
Hodgkin’s lymphoma (characterised by presence of Reid stenberg cells)
Non Hodgkin’s lymphoma
How does lymphoma typically present?
Painless lymphadenopathy
Painless, non tender ‘rubbery’ superficial lymph nodes (typically cervical, but also can be axillary or inguinal nodes)
Other symptoms can include night sweats, unintentional weight loss, fever, breathlessness and itching
What are the B symptoms of lymphoma?
Symptoms that are systemic
- Fever >38 degrees
- weight loss (over 10% is significant)
- night sweats
- pruritus (itching)
- lethargy
How is lymphoma investigated?
Lymph node excision biopsy
Staging CT scan (or PET scan)
Chest X-ray
Bloods - FBC, blood film, ESR, LFT, LDH, urate, calcium
How is lymphoma staged?
The Ann Arbor system
Stage I - confined to single lymph node region
Stage II - involvement of two or more nodal areas on the same side of the diaphragm (north and south)
Stage III - involvement of nodes on both sides of diaphragm
Stage IV - spread to distant organs e/g, liver, spleen, bone marrow
Also given a letter code A or B if they have B (systemic symptoms) or not
What are the two peaks of incidence of age of Hodgkin’s lymphoma?
Age 15-30 years
Age >50 years
What are Reed-Sternberg cells?
Large cells that are multinucleated
Found in lymph node biopsies from patients with Hodgkin’s lymphoma
Cells typically have an “owls eye” appearance
What are the four classifications of Hodgkin’s lymphoma?
Lymphocyte rich (5%) Nodular sclerosing (70%) Mixed cellularity (25%) Lymphocyte depleted (<1%)
What are the risk factors for developing haematological malignancies such as lymphoma?
Reduced immunity e.g, HIV, or post transplantation
Family history
If you have an autoimmune condition e.g, rheumatoid arthritis, lupus, Sjögren’s syndrome
Infection - associated between EBV and Hodgkin’s lymphoma
Previous treatment for cancers
What are the two different grading’s of non-hodgkins lymphoma ?
Give an example for each
Indolent (slow growing) - follicular lymphomas High grade (quick growing) - diffuse large B cell lymphomas
How do the different grades of non Hodgkin’s lymphoma react differently to chemotherapy?
Indolent (slow growing) - respond well to chemotherapy but are rarely cured
High grade (fast growing) - these are more likely to be cured with chemotherapy
What are the two main types of non-Hodgkin lymphoma and what type are they?
They are B cell line lymphomas:
Diffuse large B cell lymphoma - high grade lymphoma
Follicular lymphoma - indolent lymphoma
How do lymphomas differ from lymphocytic leukaemia in their origins?
Lymphomas are solid tumours involving the lymph nodes
Lymphoid leukaemias originate in the bone marrow and therefore typically involve circulating lymphocytes
What other type of cancer are patients with treated with chemotherapy and radiotherapy for Hodgkin’s lymphoma at risk of developing?
Lung cancer
This risk is increased x20 in smokers
What is leukaemia?
Malignant neoplastic process involving one of the white blood cell lines (neutrophils, lymphocytes, monocytes)
What are the 4 main types of leukaemia?
2 acute, 2 chronic
Acute lymphoblastic leukaemia (ALL)
Acute myeloblastic leukaemia (AML)
Chronic lymphocytic leukaemia (CLL)
Chronic myeloid leukaemia (CML)
What does the finding of pupura signify?
Purpura is the appearance of non-blanching red spots on the skin. They are caused by small areas of bleeding under the skin. Usually purpura are <1cm
What differentials should you consider if a young person presents with purpura
TTP - thrombotic thrombocytopenic purpura
HUS - haemolytic uraemic syndrome
Infections - such as meningococcal sepsis
Haematological malignancies - such as leukaemias
What is multiple myeloma?
A type of bone marrow cancer
Malignancy arising from the plasma cells (the cells responsible for antibody production)
What are the symptoms of multiple myeloma?
Bone pain (usually in the back) - due to osteolytic bone lesions
Bone fractures
Hypercalcemia
Anaemia and thrombocytopenia - proliferation of plasma cells results in reduced erythropoiesis (reduced production of platelets and RBCs)
Unusual bleeding