Haematology Flashcards
What cells do NHL arise from
B cells (85%)
T cells
Natural killer cells
How is NHL differentiated from HL
Absence of Reed-Sternberg cells at biopsy
Which type of lymphoma is more common
NHL
Most common Sx of NHL
Asymmetric painless lymphadenopathy
Features of Waldenstroms macroglobinaemia (low grade B cell neoplasm)
Raised plasma viscosity
Raised total protein
Assosiated IgM paraprotein
Can lead to hyperviscosity syndrome
How to distingush between Haemophilia A carrier and Von Willebrands in a lady
NORMAL BLEEDING TIME in Haemophilia A
Glucose 6 dehydrogenase (G6PD) deficiency predisposes patients to haemolysis when exposed to what?
Dapsone
Primaquine
Aspirin
Quinolones
Nitrofurantoin
Fava beans
Ingestion of napthalene (found in mothballs)
Hep A and B
CMV
Pneumonia
Typhoid
WHat is acquired haemolytic anaemia is a recognised complication of what?
Methyldopa
Penicillin
Quinine
Quinidine
What can myelofibrosis change into?
AML
Features of idiopathic myelofibrosis
Extensive bone marrow scelrosis - excessive fibroblast proliferation and collagen deposit in the bone marrow
Leukoerythoblastic picture
Splenomegaly
Tear droped shaped red blood cells
What is paroxysmal nocturnal haemoglobuinuria (PNH)?
Rare type of acquried haemolysis caused by intrinsic red cell abnormalities
Classic triad of paroxysmal nocturnal haemoglobuniruia
Haemolytic anaemia
Pancytopenia
Large vessel thrombosis
Genetics of PNH
Lack of complement regulatory GPI and anchored proteins (C55 and CD59) due to a a haematopoietic stem cell fault of the PIGA gene, on chromosome X
gold standard for diagnosis of PNH
Flow cytometry or fluorescent aerolysin (blood immunophenotyping)
First line Tx of PNH
Eculizumab (monoclonal Ab) for lifelong Tx
Stem cell transplant
What is shown in the urinalysis of PNH
Haemoseridin
Causes of thrombocytosis
Autonomous / primary
Secondary
- Anaemia
- Infection
- Non infectious inflammation (malignancy, rheumatological conditions, reactions to meds)
- post splenectomy
What are the most common subtypes of cutaneous T cell lymphomas
Sezary syndrome
Mycosis fungoides
WHat part of the coagulation cascade is PT in
ExtrinsicW
What part of the coagulation cascade is APTT in
Instrinsic
What is DIC
Characteristed by innapropriate systemic activation of the coagulation cascade, leading to small and medium vessel thrombosis and subsequent organ dysfunction
This can lead to major haemorrhage due to exhaustion of clotting factors and platelets
What is DIC caused by
As a complication of an underlying pathology, rather than being a primary event itself
Common causes of DIC
Sepsis
Malignancy
Incompatible blood transfusion
Obstetric emergencies
Liver failure
Widespread tissue damage from severe burns / trauma
Bloods findings in DIC
Prolonged APTT
Prolonged PT/INR
Prolonged thrombin time
Low fibrinogen
High fibrin degredation products
Low platelets
Raised d dimer
Treatment of DIC
FFP
Cyroprecipitate
Factor concentrates
What is the most common form of inherited haemolytic anaemia
Hereditary spherocytosis
Lab features of hereditary spherocytosis
Prescence of spherocytes on blood smear
Negative direct anti-globulin tests
Elevated reticulocytes
Pathology of hereditary spherocytosis
Inherited defect in structural proteins in red blood cell membranes
Abnormalities result in changes in red cell shape due to defects in skeletal proteins
Become spherical - flexibility decreases and they are removed to the spleen due to frailty
Increased red cell destruction causes hyperbilirubinaemia, elevated reticulocyte count and splenomegaly
Inheritance of G6PD
X linked recessive
Presentation of G6PD
Usually asymptomatic until a trigger causes haemolysis
Diagnosis of G6PD
Measuring G6PD activity
What is seen in the bloods during an attack of a patient with G6PD
Raised reticulocytes
Increased unconjugated bilirubin
Increased LDH
Low haptoglobin
Heinz bodies
Bite cells
Blister cells
CML causes what in the blood
Increase in neutrophils and myelocytes
What should be done if you have developed an above the knee DVT in pregnancy
LMWH for subsequent pregnancies
Ingestion of what aids iron absorption?
Vitamin C
What is aplastic anaemia
Pancytopenia with hypocellular bone marrow in the abscence of abnormal infiltrate (dysplasia, blasts) or marrow fibrosis
Presentation of asplastic anaemi
Neutropenia
- recurrent infections
anaemia
thrombocytopenia
- bleeding/bruising
Persistent warts
Bone marrow biopsy of aplastic anaemia
Hypocellular bone marrow
Treatment of aplastic anaemia
Immunosuppressants
Haematopoieitic stem cell transplantation
Most common congential cause of aplastic anaemia
Fanconi anaemia
Pathology of aplastic anaemia
Deficiency in haematopoeitic CD34+ stem cells
Presentation of fanconis anaemia
Hearing loss
Pigmentation abnormalities
Urogenital abnormalities
Splenomegaly - which produces this - myelofibrosis or myelodysplasia?
Myelofibrosis
The two major causes of gross splenomegaly
CML
Myelofibrosis
WCC in CML
often > 100 x109
Blood film of myelofibrosis
Leucoerythroblastic blood picture
Teardrop cells
Genetics of von willebrand disease
Mutation in the von willebrand gene
Screening tests for von willebrands disease
vWF antigen (diagnostic if <0.3)
vWF activity (functional assays)
Factor VIII activity
Blood tests of von willebrands disease
Normal FBC
Normal platelets / mild thrombocytopenia
Normal or mildly prolonged APTT
Normal PT
Treatment of type I + II vwd
Desmopressin (DDAVP)
Antifibrinolytics can be used in addition therapeutically or prophylactically
Treatment of type III vwd
vWF-containing concentrates
for elective surgery =- need vwF concentrate at least 8 hours pre procedure
Bleeding time in haemophilia A and B
NORMAL
Features of waldenstorms macroglobuniaemia
Monoclonal IgM peak
Lymphadenopathy
Hepatosplenomegaly
Hyperviscotiy
What is waldenstorms macroglobuniaemia
A low grad lymphoma
What indicates a poor prognosis in ALL
Prescence of philadelphia chromosome
Definitive diagnosis of ALL
20% or more of lymphoblasts in the bone marrow
What is shown in a peripheral blood smear for ALL
Peripheral blasts cells
The combination of anaemia and jaundice should indicate what until proven otherwise?
Haemolytic anaemia
A reticulocytosis indicates what?
Bone marrow is workign
WHat does anaemia in the prescence of reticulocytes indicate?
Blood cells are being lost/destroyed outside of the bone marrow
i.e. bleeding or haemolysis
If red cells show osmotic fragility, what does this indicate
Hereditary spherocytosis
When is it common to see a prolonged PT
Patients in liver disease
leading to failure of synthesis of vitamin K dependent clotting factors
Blood features of anti-phospholipid antibody syndrome
Thrombocytopenia
Positive VDRL test
Positive anti-cardiolipin Abs
Prolonged APTT
Normal PT
What is the most common cause of aplastic crisis in patients with sickle cell disease
Human parovirus B19
Definitive cure for sickle cell disease
Stem cell transplanation (reserved for patients < 17 who have severe disease non responsive to medical therapy)
WHat can sickle cell crisis be triggered by
Infection
Stress
Trauma
Pregnancy
Surgery
Cold weather
Alcohol
Hypoxia
Dehydration
Acidosis
Inheritance of sickle cell disease
AR
What should patients with sickle cell disease be started on for lifelong
Penicillin as prophylactic Abx
What may be used to prevent acute painful crisis and acute chest Synrome
Hyroxycarbamide
Where is the gene deletion with alpha thalassaemia
chromosome 16
where is the gene mutation with beta thalassaemia
chromosome 11
What do the thalassaemias result in
Haemolysis
Blood film of alpha thalassaemia
Mexican hat cells
Blood film of sickle cell anaemia
Sickle cells
Blood film of G6DH deficiency
Heinz bodies
Blister cells
Blood film of microangiopathic haemolysis
Schistocytes
Blood tests of thalassaemias
Microcytic anaemia
May be assosiated raised WCC
Reduced platelets
Serum iron and ferritin elevated
What is polycythaemia vera characterised by
Eyrthrocytosis
Thrombocytosis
Leuckocytosis
Splenomegaly
Risks of PV
Thrombosis
Haemorrhage
Transformation to acute leukaemia
Myelofibrosis
Diagnosis of PV
Increased Hb or haematocrit AND
JACK2 mutation AND
Bone marrow biopsy - hypercellularity for age with trilineage growth
Treatment of PV
Phlebotomy
Low dose aspirin
Cytoreductive therapy
Presentation of PV
HTN
Headache
Erythromelalgia (burning cyanosis in hands/feet)
Transient visual disturbance
Features of thrombosis
Gouty arhtiris (increased RBC turnover)
Bleeding
Aquagenic pruritis (itching worse with warm water)
Facial plethora
Splenomegaly
Bloods of PV
High Hb
High hct
High WCC
High Platelets
Low MCV
Decreased EPO levels
JAK2 mutation
Treatment of PV
Low dose aspirin
Phlebotomy
Management of CV risk factors
Some patients - cytoreductive therapy (hyroxyrua)
Scoring system of multiple myeloma uses what
serum B2 microglobulin
Albumin
Top two most common malignancies seen after transplant
- Skin malignancy
- Lymphoma
Why are patients with CLL immunodeficienct
Immunoglobulin deficiency
What does hepatosplenomegaly in haematology conditions indicate?
Lymphoproliferative disorder
Whats hypochromia
Reduction in amount of Hb in each RBC
WHat is usually found in the peripheral blood if you have thalassaemia
Nucleated red blood cells