KEY HAEMATOLOGY Flashcards
State the symptoms of leukaemia that can occur due to bone marrow failure.
Anaemia symptoms
thrombocytopenia (bleeding + bruising)
Neutropenia (increased risk of infections)
State the symptoms of leukaemia that can occur due tissue infiltration.
Bone pain Gum hypertrophy Hepatosplenomegaly Lymphadenopathy Cranial nerve palsies Testicular enlargement Mediastinal mass
State the symptoms of leukaemia that can occur due to substance release.
DIC (bleeding + bruising)
Hyperuricaemia (gout, renal stones, AKI).
Name the 4 groups of myeloid malignancies.
AML
CML
Myelodysplastic syndrome
Myeloproliferative neoplasms
Who is AML more common in?
Older adults + the elderly.
The presence of which cells in the blood stream is always abnormal?
**They indicate AML.
Blast cells.
What can AML occur secondary to?
Myelodysplastic syndromes.
Name the main subtype of AML.
Acute promyelocytic leukaemia.
What mutation is acute promyelocytic leukaemia due to?
15:17 translocation.
What cells are pathognomonic of AML/ acute promyelocytic leukaemia?
Auer rods.
General FBC findings in AML?
Pancytopenia.
Low Hb, low WCC, low plts.
Blood film findings in AML?
Blast cells (myeloblasts) Auer rods
Bone marrow aspirate findings in AML?
Increased cellularity
Replacement by blast cells of >20%
1st line treatment for acute promyelocytic leukaemia?
All-trans-retinoic acid + chemotherapy.
Acute promyelocytic leukaemia commonly presents as what?
DIC.
Blast cells will stain positive for what in AML?
Myeloperoxidase.
What causes are associated with AML?
Radiotherapy + alkylating agents
Condition associated with development of AML/ CML?
Down’s syndrome.
All leukaemia cells in CML contain what?
The Philadelphia chromosome.
What is the Philadelphia chromosome?
9:22 translocation forming BCR-ABL gene.
What can CML turn into?
AML.
Most common physical finding in CML?
Splenomegaly.
FBC findings in CML?
Could be normal.
If anything will cause neutrophilia.
**Classically = isolated raised eosinophils or basophils.
Blood film findings in CML?
Leukoerythroblasts.
Bone marrow aspirate findings in CML?
Increased cellularity.
1st line treatment for CML?
Imatinib.
General overall findings in myelodysplastic syndrome.
Blood cytopenias + hyper cellular bone marrow.
Myelodysplastic syndromes can predispose to what?
AML.
In myelodysplastic syndromes, morphological abnormalities are present in how many cell lines?
2 or more.
FBC findings in myelodysplastic syndromes.
Low RBCs ± low WCCs ± Low platelets.
How are myelodysplastic syndromes different to CML?
Overall neutropenia which is not seen in CML.
How are myelodysplastic syndromes diagnosed?
Bone marrow aspiration with <20% blast cells.
Management of myelodysplastic syndromes?
Mainly conservative - monitor the condition.
Name the 4 myeloprpliferative neoplasms.
Polycythaemia vera
Essential thrombocythaemia
Myelofibrosis
CML
Why are the myeloproliferative neoplasms grouped together?
They all have the ability to transition from one disease to another and can all transform into AML.
Mutation seen in >95% of those with polycythaemia vera.
JAK2.
Difference between polycythaemia vera and secondary polycythaemia?
Secondary polycythaemia has no splenomegaly.
Management of polycythaemia vera.
Venesection + chemotherapy.
Symptoms of polycythaemia vera.
Tiredness, itching, vertigo, headache, visual disturbances.
**All due to the expanded cell volume.
When does the itching associated with polycythaemia vera tend to be worse?
After a hot bath or shower.
Problem in polycythaemia vera?
Too many red blood cells.
Problem in essential thrombocythaemia?
Too many platelets.
Mutation present in >50% patients with essential thrombocythaemia?
JAK2.
Symptoms of essential thrombocythaemia?
Bruising, bleeding. cerebrovascular symptoms.
Management for essential thrombocythaemia?
Treat if symptoms present.
Hydroxyurea + aspirin used.
4 characteristics of myelofibrosis.
1) Fibrosis in bone marrow
2) Extramedullary haematopoiesis
3) Anaemia
4) Tear drop poikilocytosis in peripheral blood
> 50% those with myelofibrosis have what mutation?
JAK2.
Main sign in those with myelofibrosis?
Massive splenomegaly.
Finding on peripheral blood film in myelofibrosis?
Tear drop poikilocytosis.
General underlying pathophysiology in myelofibrosis?
Fibrosis of the bone marrow causing extramedually haematopoiesis.
4 main causes of massive splenomegaly?
CML
Myelofibrosis
Polycythaemia vera
Chronic TB
ALL normally causes massive proliferation of what type of cell?
B cells.
ALL is most common in who?
Young children.
ALL is associated with which condition?
Down’s syndrome.
Common symptoms and signs in ALL?
Mediastinal mass ± respiratory symptoms.
ALL is more likely than other forms of leukaemia to affect which system?
CNS.
FBC findings in ALL.
Very low myeloid cells.
High lymphocyte count.
Blood film findings in ALL?
Lymphoblast cells.
Bone marrow aspirate findings in ALL?
Hypercellularity with >20% blast cells.
Investigations often needed in ALL?
CXR (mediastinal mass?)
LP (any abnormal cells in CNS?)
Management of ALL?
Chemotherapy + intrathecal prophylactic chemotherapy.
If abnormal cells found on LP, cranial irradiation may be needed.
What is the most common leukaemia?
CLL.
What sign occurs in 80% patients with CLL?
Lymphadenopathy.
What is Richter’s transformation?
Where CLL changes into diffuse large B cell lymphoma.
Findings on FBC for CLL?
Raised WCC.
Blood film findings in CLL?
Smear/ smudge cells.
Bone marrow aspirate findings in CLL?
Hypercellularity with blast cells.
Condition associated with CLL?
Warm autoimmune haemolytic anaemia.
Most common cause of death in CLL?
Infection (naive B cells do not work).
Myeloma is malignancy of what cells?
Plasma cells (causes production of large amounts of abnormal antibodies)
Antibody normally produced in myeloma?
IgG.
Prevalence of myeloma increases in proportion to what?
Increasing age.
Precursor to myeloma?
MGUS.
Stage between MGUS and myeloma?
Smouldering.
Name the common features of myeloma.
1) Calcium high - bone destruction.
2) Renal impairment - commonly damaged by light chain nephropathy.
3) Anaemia - marrow infiltration + CKD.
4) Bone disease + pain - lytic lesions due to increased osteoclastic activity.
5) Spinal cord compression.
FBC findings in myeloma.
Pancytopenia.
Blood film finding in myeloma?
Rouleaux formation.
Plasma cells >10%.
Special test for myeloma?
Urine electrophoresis + serum electrophoresis for Bence-Jones proteins
Prognostic indicator for myeloma?
Beta-2 microglobulins.
Condition similar to myeloma which causes release of IgM antibodies?
Waldenstrom’s macroglobulinaemia.
Other blood findings in myeloma?
High calcium
High ESR
Skull imaging finding in some patients with myeloma?
Raindrop/ Pepperpot skull
2 types of lymphoma?
Hodgkin’s + Non-Hodgkin’s
Most common subtype fo lymphoma?
Non-Hodgkin’s
Common presentations for lymphomas.
Lymphadenopathy + B symptoms.
What are B symptoms?
Systemic symptoms of:
- Fever
- Night sweats
- Weight loss (>10% in 6/12)
Hodgkin’s lymphoma affects what kinds of people?
Tends to affect younger people.
Lymph node biopsy findings in Hodgkin’s lymphoma?
Reed-Sternberg cells.
Most probable cause of Hodgkin’s lymphoma?
EBV.
Staging used for Hodgkin’s lymphoma?
Ann-Arbor staging
Ann Arbor stage 1?
Problem in 1 lymph node or group of nodes.
Ann Arbor stage 2?
Problem in 2 places on same side of diaphragm.
Ann Arbor stage 3?
Malignant problem on both sides of diaphragm.
Ann Arbor stage 4?
Involvement of extra-nodal sites.
What is Pel Ebstein fever?
Cyclical fevers occurring over a few weeks.
Pel Ebstein fever is associated with what?
Hodgkin’s lymphoma.
Which subtype of Non-Hodgkin lymphoma is associated with EBV?
Burkitt’s lymphoma (worst prognosis).
Most common adult lymphoma?
Diffuse large B cell lymphoma.
Lymphoma with worst prognosis?
Burkitt’s.
Lymphoma affecting the stomach, associated with H. Pylori?
MALT.
Blood film finding in Burkitt’s lymphoma?
‘Starry-sky appearance’.
Increased itch after heat exposure in polycythaemia probably caused by what?
Histamine ± prostaglandin release.
Indicators of a poor prognosis in Hodgkin’s lymphoma.
Male gender B symptoms present Lymphocyte depleted Fever Age >45 at diagnosis
What might have happened if new B symptoms are developed in a patient with CLL?
Richter’s transformation.
Empirical antibiotic in neutropenic sepsis?
Pip-Taz.
Polycythaemia vera has the capacity to transform into what conditions?
Myelofibrosis/ AML.
Most common type of Hodgkin’s lymphoma?
Nodular sclerosing.
Subtype of Hodgkin’s lymphoma with best prognosis?
Lymphocyte predominant Hodgkin’s lymphoma.
Overall most common type of lymphoma?
Diffuse large B cell lymphoma.
What do Howell-Jolly bodies suggest?
Hyposplenism (can be seen in coeliac disease).
Blood film anomaly seen in CML?
Pseudo-pelger-huet anomaly.
Haematological malignancy associated with Rouleaux formation?
Myeloma.
Haematological malignancy associated with Reed-Sternberg cells?
Hodgkin’s lymphoma.
Type of Hodgkin’s lymphoma with worst prognosis?
Lymphocyte depleted.
Polycythaemia vera causes what FBC finding?
Isolate rise in RBC count.
Thymomas are strongly associated with what condition?
Myasthenia Gravis.
