Haematology Flashcards
How long does it take to produce a neutrophil?
7-10 days
What is the quickest response for releasing neutrophils?
Demargination
Give some causes of neutrophilia
Infection/inflammation Steroids, adrenaline, GCSE Cold/heat/surgery Myeloprofilerative disorder Malignancy
What is the lifespan of a neutrophil?
12-18 hours
What is GCSF used for?
It stops the WCC dropping too low during chemotherapy
Therefore can be a cause of neutrophilia
Give some causes of neutropenia
Chemotherapy agents Anticonvulsants, carbimazole, IBD meds Viral infection BM failure Immune mediated - SLE
Give some causes of lymphocytosis
Viral infections: EBV, CMV, influenza Leukaemia, lymphoma MI Sickle cell crisis Trauma RA Splenectomy
What is the commonest cause of a >6 week lymphocytosis?
Chronic lymphocytic leukaemia
Give some causes of a lymphopenia
Malignancy - Hodgkin’s or NHL RA, SLE Chemotherapy Burns Anorexia nervosa Renal or liver failure Sarcoidosis Aplastic anaemia Myelodysplatic syndromes
Give some causes of eosinophilia
Allergic conditions:asthma, eczema, hayfever
Allergic reaction to medication
Parasites; tapeworm, hookworm, schistosomiasis
Hodgkin’s lymphoma
Sarcoidosis
Irradiation
What is aquagenic pruritus?
Itching when exposed to hot water
What is polycythaemia Vera?
All blood cell types are produced excessively
Particular increase in the RBCs
This is a myeloproliferative neoplasm
Linked with JAK2 mutation
What do smear cells indicate?
Chronic lymphocytic leukaemia
Define leukaemia
A group of disorders that are characterised by the accumulation of malignant white cells in the bone marrow (primarily) and peripheral blood
How can we tell if BM failure is acute or chronic?
Mature cells present if chronic
Immature cells present if acute
Is CLL curable?
No
CLL is a disorder of which cells?
B cells
B1
How might CLL present?
Insidious onset Recurrent infections Enlarged LNs Mucocutaneous bleeding Tiredness and fatigue
Are B symptoms common in CLL?
No
What are the B symptoms?
Fever
Weight loss
Night sweats
What degree of weight loss is considered worrying?
> 10% in the last 6 months
Give a differential diagnosis for low Hb, low WCC and low platelets
Acute leukaemia until proven otherwise : ALL/AML
Aplastic anaemia
B12 deficiency
What is the purpose of flow cytometry?
To see if the lineage is lymphoid or myeloid
What blood tests need doing if you suspect DIC?
PT
APTT
Fibrinogen
How many days from diagnosis do you have to treat a haematological malignancy?
7 days
What are the signs of leukostasis?
Respiratory distress
Decreased mental status
Priapism
What gene diagnoses CML and how do we detect it?
BCR-ABL
FISH
What disease is the Philadelphia chromosome linked to?
Chronic myeloid leukaemia
What is the management of CML?
Tyrosine kinase inhibitor
BM transplant
What age groups get Hodgkin’s lymphoma?
15-30 years
>60 years
What proportion of patients have B symptoms in Hodgkin’s lymphoma?
Third
What other disease is Hodgkin’s linked to?
EBV exposure
What is the prognosis for Hodgkin’s lymphoma?
Good
80% cure
What is the management of Hodgkin’s?
Radiotherapy
Chemotherapy ABVD combo
What medicines are in the ABVD combo?
Adriamycin (doxorubicin)
Bleomycin
Vinblastine
Dacarbazine
Describe the Ann Arbor Staging
I = 1 LN region II = 2 + LN regions III = 2+ LN regions on different sides of diaphragm IV = extralymphatic spread
What are the classic 3 signs of myeloma?
Anaemia
Back pain
High ESR
Myeloma makes what type of bone lesions?
Lytic
How do we diagnose myeloma?
Histology >10% plasma cells in BM
Paraprotein or excess light chains
Myeloma related organ dysfunction
What is the management of myeloma?
Steroids - dexamethasone
Chemo - cyclophosphamide
Thalidomide
Proteasome inhibitors
What is the CRAB mnemonic for myeloma?
C - calcium increase
R - renal insufficiency
A - anaemia
B - bone lesions
Why can tinnitus occur in severe anaemia?
Hyperdynamic circulation
Name some causes of microcytic anaemia
Iron deficiency Anaemia of chronic disease Thalassaemia Hereditary spherocytosis Sideroblastic anaaemia Hodgkin’s disease
What is dysfunctional in haemochromatosis?
DMT 1 transporters are overzealous in the duodenum
What is ferroportin?
A protein which dictates how much iron is brought into the circulation
What cells recycle iron from RBCs?
Macrophages
How is iron stored?
2/3 in RBCs
Intracellular ferritin in Spleen, liver and bone marrow
Myoglobin
Iron containing enzymes
What is the natural iron turnover in a day?
1 mg
What is the maximum iron that can absorbed from a diet?
20-25 mg/ day
What will a blood film show in iron deficiency anaemia?
Variable RBC size and shape
Microcytes
Hypochromia
Target cells
Why might ferritin be increased in an iron deficiency anaemia?
Ferritin is an acute phase reactant and therefore can by raised with acute inflammation
What are the non-megaloblastic causes of macrocytosis?
Chronic alcohol excess Liver disease Haemolysis Hypothyroidism Pregnancy
What are the megaloblastic causes of macrocytosis?
B12 or folate deficiency
Primary bone marrow disorders eg. Myelodysplasia
Drugs interfering with DNA synthesis eg. Azathioprine
What does megaloblastic mean?
The nucleus is more immature than the cytoplasm
If B12 is low, what other test needs ordering?
Anti- IF
Intrinsic factor antibodies
Give some causes of B12 deficiency
Pernicious anaemia
Post total gastrectomy
Vegan
Terminal ileum disease eg. Crohn’s, worms, diverticula
How long do the body stores of B12 last?
2-3 years
What are the causes of folate deficiency?
Poor diet
Increased requirement: pregnancy, haemolysis, haemodialysis
Malabsorption
Drugs eg. Trimethoprim
How long do body stores of folate last?
3 months
What are the serious complications of B12 deficiency?
Peripheral neuropathy
Subacute combined degeneration of cord
What are the causes of normocytic anaemia?
Acute blood loss Anaemia of chronic disease Renal failure Marrow failure Hypothyroidism Pregnancy Haemolysis
Name some hereditary causes of haemolysis
Sickle cell anaemia, thalassaemia
G6PD deficiency
Hereditary spherocytosis, elliptocytosis
Name some acquired causes of haemolysis
Infections via complement activation
Physical damage eg. DIC
Drug induced
Autoimmune
What is included in a haemolysis screen?
FBC, reticulocyte count, blood film Direct Coombs test Bilirubin (unconjugated) LDH Haptoglobin
What is the role of haptoglobin?
To mop up free Hb
Beta 2 microglobulin is a tumour marker for …
Myeloma
CLL
some lymphomas
What gene do we look for in CML?
BCR - ABL
What is the tumour marker for non Hodgkin lymphoma?
CD20
What are the haem red flags?
Anaemia Persistent or recurrent infections Fever Night sweats Neck lumps Unexplained bleeding or bruising Bone pain Pruritus
Describe Ann Arbor staging
I : 1 LN group involved
II: 2 LN groups on same side of diaphragm
III: >2 LN groups on both sides of diaphragm
IV: extralymphatic organ involvement
Name some causes of mild splenomegaly
Acute splenitis
Acute congestion
Infectious mononucleosis
Acute febrile disorders
Name some causes of moderate splenomegaly
Chronic congestion Acute leukaemia Hereditary spherocytosis Thalassaemia major AI haemolytic anaemia Amyloidosis
Name some causes of massive splenomegaly
Chronic myeloproliferative disorders CLL Hair cell laeukaemia Lymphomas Malaria Primary splenic neoplasms
What can MGUS turn into?
Myeloma
What is DIC?
Clotting system becomes overactive and clots form in the small vessels. This uses up all the clotting factors so it can lead to excessive bleeding
Name some causes of DIC
Infections (sepsis)
Cancer
Trauma/major surgery
Pregnancy and childbirth
What percentage of ALL occurs in children?
60%
What percentage of AML occurs in children?
20%
What percentage of CML associated with BCR ABL?
> 90%
Name some risk factors for AML development
Prolonged exposure to benzene and petroleum
Prior chemotherapy with alkylating agents
Down syndrome
Bloom syndrome
Fanconi anaemia
How does leukaemia present?
Fatigue
Bruising or bleeding esp mucosal surfaces
Fever
Infections
What complications can hyperviscosity lead to?
Priapism
Tinnitus
Retinal haemorrhage
CVA
What is the treatment for acute leukaemia?
Combination chemotherapy to achieve remission
Supportive therapies eg. Abx, RBCs, platelets
Broadly, how is CLL treated?
Only treat if Sx arise
Chemotherapy
Steroids
Rituximab
What percentage of patients with Hodgkin’s have a normal lifespan?
80
When are the peaks for Hodgkin’s?
20s and 60s
What is the most common subtype of Hodgkin’s?
Nodular sclerosing
What investigations would you order for suspected Hodgkin’s?
FBC, ESR, U+E, LFT, ALP,LDH
Biopsy of involved LN
CXR and CT CAP
Consider BM biopsy
What is the treatment of Hodgkin’s?
Stage 1-2a radiotherapy
Stage 2b + chemotherapy
What is the combination chemo given for Hodgkin’s?
ABVD
Adriamycin, bleomycin, vinblastine, dacarbazine
What factors would indicate a poor prognosis in Hodgkin’s?
B symptoms >45 years old Bulky mediastinal disease Extranodal involvement Decreased haematocrit or increased ESR Increased CD30 and IL2
What percentage of patients with Hodgkin’s have a normal lifespan?
80
When are the peaks for Hodgkin’s?
20s and 60s
What is the most common subtype of Hodgkin’s?
Nodular sclerosing
What investigations would you order for suspected Hodgkin’s?
FBC, ESR, U+E, LFT, ALP,LDH
Biopsy of involved LN
CXR and CT CAP
Consider BM biopsy
What is the treatment of Hodgkin’s?
Stage 1-2a radiotherapy
Stage 2b + chemotherapy
What is the combination chemo given for Hodgkin’s?
ABVD
Adriamycin, bleomycin, vinblastine, dacarbazine
What factors would indicate a poor prognosis in Hodgkin’s?
B symptoms >45 years old Bulky mediastinal disease Extranodal involvement Decreased haematocrit or increased ESR Increased CD30 and IL2
What is the median age of presentation of NHL?
50+
What investigations would you do for suspected NHL?
Excisional biopsy FBC, U+E, LDH, Beta 2 microglobulin BM aspirate/trephine CXR CT CAO
What is the management of low grade NHL?
Radiotherapy
Chemotherapy - cyclophosphamide, vinocristine
Prednisolone
Rituximab
What is the management for intermediate or high grade NHL?
Radiotherapy and combination chemo
CHOP
What is myeloma?
A clonal proliferation of mature plasma cells that secrete Ig leading to an overproduction of a single Ig class (paraprotein)
What is the management of myeloma?
Correct any deficits Allopurinol Opiates and RT for bone pain Chemo: cyclophosphamide, thalidomide and dexamethasone Stem cell transplant
What is the 5 year survival of myeloma?
35%
What is Waldenstroms macroglobulinaemia?
Rare type of slow growing NHL
Lymphoplasmacytic cells become abnormal and can build up in LNs, BM or Spleen
Makes large amounts of IgM
Was is included in RCHOP therapy?
Rituximab Cyclophosphamide Doxorubicin (hydroxy..) Vincristine (oncovin) Prednisolone
What is RCHOP chemo used for?
Non-Hodgkins lymphoma
What is included in ABVD chemo?
Doxorubicin (adriamycin)
Bleomycin
Vinblastine
Dacarbazine
What is ABVD chemo used for?
Hodgkin lymphoma