Haematology Flashcards
What is pancytopenia?
Low RBC + Low WBC + Low Platelets
- think malignancy
Raised MCV + Raised bilirubin consider what…
Haemolytic process!
What is seen on a haemolytic blood test screen?
Haptoglobin low <0.03, LDH raised, DAT + Ve (Coombs test), Raised reticulocytes 330 (25-75)
How should haemolysis be managed?
- Call haematology (it’s an emergency).
- Start folic acid
- Start steroids
What is a positive Coombs test?
An abnormal (positive) direct Coombs test means you have antibodies that act against your red blood cells. This may be due to: Autoimmune hemolytic anemia. Chronic lymphocytic leukemia or similar disorder.
Epidemiology of acute lymphoblastic leukaemia
Acute lymphoblastic leukaemia (ALL) is the most common malignancy affecting children and accounts for 80% of childhood leukaemias. The peak incidence is at around 2-5 years of age and boys are affected slightly more commonly than girls.
What is the difference between myeloid and lymphocytic leukaemias?
Myeloid comes from myeloid precursors (neutrophils)
Lymphocytic comes from lymphoid precursors (B cells)
What is ALL?
Acute lymphoblastic leukaemia
ALL is the most common cancer of childhood (2-5yrs peak). It is caused by the abnormal clinical proliferation of lymphoid progenitor cells. These lymphoid precursors infiltrate normal haematopoietic cells of the bone marrow and other organs of the body.
Chronic myeloid leukaemia (CML) epidemiology and associations
CML is most common in middle-aged patients, with males slightly more affected.
It is classically associated with the Philadelphia chromosome.
What is CLL?
CLL is most common in male patients over the age of 60. It is caused by the proliferation of functionally incompetent malignant B cells.
What is the difference between acute vs chronic leukaemia’s?
The difference between acute and chronic leukaemia is that acute leukaemia is a result of impaired cell differentiation, resulting in large numbers of malignant precursor cells in the bone marrow; on the other hand, chronic leukaemia is the result of excessive proliferation of mature malignant cells, but cell differentiation is unaffected.
How does AML present?
It typically presents with symptoms of bone marrow failure (anaemia, bleeding, infections) and signs of infiltration, including: hepatomegaly, splenomegaly, and gum hypertrophy.
How is AML diagnosed?
Blood tests commonly show leucocytosis, but white cells can sometimes be normal or low. For this reason, diagnosis is dependent on bone marrow biopsy, as well as other molecular analyses.
What is seen on bone marrow biopsy in AML?
Auer rods
Treatment of AML:
AML is commonly treated with chemotherapy regimens or bone marrow transplant.
AML prognosis:
Without treatment: Dead in 2 months
With treatment: 20% 3-year survival rate.
Pathophysiology of ALL - read to understand
Abnormal proliferation of lymphoid progenitor cells. Lymphoid precursors infiltrate normal haematopoietic cells of the bone marrow and other organs of the body.
ALL epidemiology
2-5yrs peak!
(Accounts for 80% of childhood leukaemia’s).
Symptoms of ALL
Bone marrow failure = pancytopenia = anaemia, bleeding, infections).
Symptoms may also be caused by organ infiltration, such as bone pain.
Signs on examination in ALL
Signs include painless lymphadenopathy, hepatosplenomegaly, CNS involvement (e.g. cranial nerve palsies, meningism) or testicular infiltration (resulting in painless unilateral testicular enlargement).
Diagnosis of ALL
Blood results show leucocytosis and blast cells on blood film and bone marrow analysis.
Treatment and prognosis of ALL
Chemo
Children have a cure rate of 70-90%.
CML pathophysiology
Proliferation of functionally incompetent malignant B cells.
Philiadelphia chromosome translocation.
- commonly pt aged 60-70
Symptoms of CML?
Symptoms: weight loss, tiredness, fever, and sweating, bleeding (thrombocytopenia), and gout.
What sign is key in CML on examination?
Massive splenomegaly (>75%).
Diagnosis of CML
Bloods: Leucocytosis, in particular raised myeloid cells which include: neutrophils, monocytes, basophils, and eosinophils
1st line tx for CML
Imatinib
Pathophysiology of CLL
Proliferation of functionally incompetent malignant B cells.
Presentation of CLL
Often asymptomatic!
B symptoms (weight loss, night sweats, and fever).
Signs: Non-tender lymphadenopathy, hepatosplenomegaly.
Pancytopenia symptoms due to bone marrow failure is uncommon.
Diagnosis of CLL
Incidental lymphocytosis!
Smudge cells
What is myeloma?
Cancer of the plasma cells.
Explain MGUS and smouldering myeloma.
Monoclonal gammopathy of undetermined significance(MGUS) may predispose.
Smouldering myeloma is when MGUS has progressed and is consider premalignant.
When to suspect multiple myeloma in a GP setting and how to approach with initial investigations?
Consider myeloma in anyone over 60 with persistent bone pain, particularly back pain, or an unexplained fractures.
Initial Investigations:
* FBC(low white blood cell count in myeloma)
* Calcium(raised in myeloma)
* ESR(raised in myeloma)
* Plasma viscosity(raised in myeloma)
Multiple myeloma symptoms
- C–Calcium (elevated)
- R–Renal failure
- A–Anaemia (normocytic, normochromic) from replacement of bone marrow.
- B–Bone lesions/pain
Blood findings in myeloma
Anaemia
Renal impairment (particularly raised serum creatinine)
Hypercalcaemia
What does bone marrow biopsy show in myeloma?
Tissue diagnosis typically by bone marrow aspirate and biopsy: myeloma is confirmed if there are >10% of plasma cells in the bone marrow.
What complications need to be managed in myeloma and how?
- Analgesia, bisphosphonates, surgical stabilisation, and physiotherapy for bone disease.
- Influenza and pneumococcal vaccination for infection prevention.
- Erythropoietin (± transfusion) for anaemia.
Management of bone disease in myeloma:
- Myeloma bone diseasecan be improved usingbisphosphonates. These suppress osteoclast activity.
- Radiotherapyto bone lesions can improve bone pain.
- Orthopaedic surgerycan stabilise bones (e.g. by inserting a prophylactic intramedullaryrod) or treat fractures.
Investigations to diagnose myeloma:
BLIP
What additional imaging is needed in myeloma and why?
Imagingis required to assess for bone lesions. The order of preference to establish this is:
- Whole bodyMRI
- Whole bodyCT
- Skeletal survey(xray images of the full skeleton)
What signs are seen on x-ray in myeloma?
- Punched out lesions
- Lytic lesions
- “Raindrop skull” caused by many punched out (lytic) lesions throughout the skull that give the appearance of raindrops splashing on a surface
Giant multinucleated cells
Reed sternberg cells
What is diagnostic for Hodgkin’s lymphoma?
Lymph node biopsy
Reed-Sternberg cells are diagnostic: these are large cells that are either multinucleated or have a bilobed nucleus with prominent eosinophilic inclusion-like nucleoli (thus giving an ‘owl’s eye’ appearance).
Painless asymmetric lymph node swelling → think…
Hodgkin’s lymphoma
What investigations are seen in Hodgkin’s lymphoma?
Normocytic anaemia
Eosinophilia
Raised LDH
Biopsy - Reed sternberg cells
