Haematology Flashcards
Philadelphia +ve
CML
Tear drop poikilocyte
Nucleated RBC
Myelocyte
Leucoerythroblastic Anaemia
immature WBC and RBC in anaemia
Stain for reticulocytes
Methylene Blue
Hereditary Spherocytosis caused by defect in…
Spectrin
Increased LDH and haptoglobins
Haemolysis
Which cancer causes MAHA?
Adenocarcinoma (prostate)
Haemolytic Uraemic Syndrome caused by which bug?
E. coli
Auer Rods
AML
Which virus causes Adult T Cell Lymphoma
Human T-Lymphotrophic Virus 1 (HTLV1)
Adult T Cell Lymphoma in which ethnicities?
Caribbean and Japan carriers
Alcohol-induced pain
Hodgkin Lymphoma
L&H Cells (“popcorn cells”)
Nodular Lymphocyte Predominant Hodgkin = “non-classical Hodgkin)
L = lymphocytic and histiocytic cells
t(14;18) –> overexpression of bcl2 by cancerous B cells
Follicular Non-Hodgkin
H. pylori causes which NHL?
Gastric MALToma
Coeliac Disease associated with which NHL?
Enteropathy Associated T Cell Lymphoma (EATL)
Reed-Sternberg Cells
Hodgkin Lymphoma
t(11;14) –> over-express cyclin D1
Mantle Cell Lymphoma
Starry-sky appearance on lymph node biopsy
Burkitt’s Lymphoma
Burkitt’s Lymphoma caused by which virus?
EBV
Large “epitheloid” lymphocytes on lymph node biopsy
Anaplastic Large Cell Lymphoma
t(2;5) –> Alk-1 protein expression
Anaplastic Large Cell Lymphoma
Smear/Smudge Cells (fragile cells which open up = purple splodges)
Autoimmune Haemolytic Anaemia (including CLL!)
Pelger-Huet: bilobed neutrophils
Myelodysplasia
Infection causing bone marrow failure
Parvovirus
Ringed Sideroblasts: iron accumulation in ring around nucleus
Myelodysplasia (and AML)
Anaemia with abnormal thumbs and Cafe au lait spots
Fanconi Anaemia
DKC1 and TERC mutations
Dyskeratosis Congenita = reduced tolemere length
Itchy after warm water on skin
Polycythaemia Vera
Massive splenomegaly, causing Budd-Chiari Syndrome (hepatic veins draining liver are blocked)
Myelofibrosis
Skin pigmentation, nail dystrophy and leukoplakia
Dyskeratosis Congenita
JAK2 V617F mutation
Polycythaemia Vera
Myelocytes
CML
Leukaemia which is emergency as die from rapid haemorrhage/DIC
Acute Promyelocytic Leukaemia
t(9;22) in Philadelphia chromosome
Chronic Myeloid Leukaemia
Philadelphia-positive cases treated with…
Imatinib
Tear drop cell and macrocytes
Pernicious Anaemia (vit B12 and folate deficiency)
Antibodies in Pernicious Anaemia
Parietal Cell and Intrinsic Factor antibodies
Leading cause of mortality in pregnancy
VTE
Antiphospholipid Syndrome antibodies
Lupus Anticoagulant (LA) and Anti-Cardiolipin (ACL) antibodies
Test to differentiate IDA from Thalassaemia Trait
Hb Electrophoresis
- IDA: normal
- Thalassaemia: increased HbA2 in beta-thalssaemia, normal in alpha
Megakaryocytes in neonate
Neonatal Leukaemia (develops in utero and remits spontaneously)
Howell-Jolly Body
Sickle Cell Anaemia
infarction of spleen –> hyposplenism
What animal causes hyposplenism in sickle cell?
Dog bites = capnocytophaga canimorsus
Hyperplasia of facial bones
Beta-Thalassaemia
increased EPO –> bone expansion
Irregularly contracted cells
G6PD deficiency
Schistocytes
MAHA (fragmented red cells)
Coagulation disorder with NORMAL bleeding time
Haemophilia A
Universal donor
Group O, RhD –ve
Universal recipient
Group AB, RhD+ve
Maculopapular rash/desquamation after blood transfusion
Transfusion-Associated Graft Vs Host Disease
Patients affected by Post-Transfusion Purpura
HPA-1a negative patients
Polychromatic macrocytes
G6PD deficiency
100 days after bone marrow transplant, develop widespread illness that looks autoimmune.
Chronic Graft vs Host Disease
Drug which reverses heparin
Protamine
“Toxic granulation and vacuolation”
Sepsis
Koilonychia and Angular Cheilitis
Iron Deficiency Anaemia
Glossitis (“beefy tongue”)
B12 Deficiency
Target and pencil cells
Iron Deficiency Anaemia
Thrombotic Thrombocytopaenia Purpura (TTP) caused by what deficiency?
von Willebrand Factor Cleaving Protease (ADAMTS13)
Common infections in splenectomy patients
Encapsulated (pneumococcus, meningococcus)
Low platelets in post-surgical/septic patient
DIC
Osmotic Fragility Test
Hereditary Spherocytosis
Glucose-6-Phosphate Dehydrogenase Deficiency have deficiency in what?
Glutathione
G6PD helps make NADPH, which makes glutathione, which protects RBCs against oxidative stress…
Echinocytes
Pyruvate Kinase Deficiency
red cells have projections
Di-Binding Test
Hereditary Spherocytosis
Middle Eastern baby with sudden pallor and jaundice
G6PD deficient who ate fava beans
Heinz-Bodies
G6PD Deficiency
Stain for Heinz-Bodies
Methyl violet stain
Pectus excavatum, lax joints and a high-arched palate.
Ehlers-Danlos syndrome
The commonest adult leukaemia
Chronic lymphocytic leukaemia
Tan-to-grey hyperpigmented patches on his upper trunk, neck and face, nail ridges
Leukoplakia
Dyskeratosis congenita
Ham’s Test positive
also abdo pain and pancytopaenia
Paroxysmal nocturnal haemoglobinuria
19 year old Jewish male presenting with multiple pathological fractures and hypersplenism
Gaucher’s disease (glucosylceramide lipidosis)
Donath-Landsteiner positive
Paroxysmal cold haemoglobinuria
Bone marrow red cell aplasia and a positive Paul-Bunnell test
B19 parvovirus infection
Anti-leucocyte antibodies with similar specificity to the patient’s white blood cell antigens
TRALI – Transfusion related acute lung injury
Deficiency of which immunoglobulin causes anaphylaxis after blood transfusions?
IgA Deficiency
Haemolytic Uraemic Syndrome is triad of what?
- MAHA
- AKI
- Thrombocytopaenia
Low Factor VIII and low ristocetin cofactor activity
Von Willebrand Disease
Tumour cells express tartrate-resistant phosphatase (TRAP)
Hairy Cell Leukaemia
- Cancer of B cells (subset of CLL)
- Middle-aged men
Japanese person with lymphocytosis and hypercalcaemia
Tumour cells have cloverleaf appearance
Adult T-cell Leukaemia
- Caused by Human T-Lymphotrophic Virus 1
- Common in Japs and Carribeans
t(11;14) –> over-expression of BCL-2 and cyclin-D1
Mantle Cell Lymphoma
-Aggressive B cell lymphoma in old men
Tumour cells contain centrocytes and centroblasts
Follicular Lymphoma
- Centrocytes = small B cells with irregular nuclei
- Centroblasts = large B cells with many nuclei
Presents with painless “waxing and waning” lymphadenopathy
When would you give irradiated blood?
Very immunosuppressed — prevents Graft vs Host Disease (donor WBCs attack host)
Old man with vision disturbances (looks like looking out of windscreen). Protein electrophoresis shows an IgM paraprotein
Waldenstrom’s macroglobulinaemia
Sudden jaundice and haemaglobinuria after cold exposure
Paroxysmal cold haemoglobinuria
-Donath-Landsteiner antibodies
What does warfarin inhibit?
Vitamin K epoxide reductase
Which haematological cancer is increased in first 3 years in Down Syndrome?
AML –> get Auer Rods!
BCR-ABL fusion gene
CML – arises from Philadelphia Chromosome = t(9;22)
t(15;17) –> PML-RARA fusion gene
Acute Promyelocytic Leukaemia
What medical emergency is Acute Promyelocytic Leukaemia associated with?
DIC
Desmopression treats this clotting disease
Von Willebrand Disease
Bite cells
G6PD Deficiency
Basophilic stippling
Beta-Thalassaemia trait (and lead poisoning)
Acanthocytes (spiculated blood cells/spur cells)
Hyposplenism
“Turkish patient”
Turkish (G6P)Delight!
Mechanism of Factor V Leiden
Resistance to protein C
Sudden anaemia and low reticulocytes
Parvovirus
When would you give Fresh Frozen Plasma?
Person with coagulopathy is bleeding, and cannot give them the relevant factor
IgA deficient need what type of blood product?
Washed red blood cells
Lack of HPA-1a antigen causes this transfusion reaction
Post-transfusion purpura
Definition of accelerated phase in CML (in terms of % blasts)
10-19% blasts
Definition of blast crisis in CML (in terms of % blasts)
> 20% blasts
Treatment of blast crisis in CML
Allogeneic Stem Cell Transplant
3 components of Virchow’s Triad
- Hypercoagulability
- Vessel wall injury
- Stasis of blood
What measures Intrinsic and Common pathways?
APTT
Which clotting factors are in Intrinsic and Common pathways?
Factors 8 and 9 (= Haemophilia!)
What measures Extrinsic pathway?
PT
Which clotting factors are in Extrinsic pathway?
Factors 2, 7 and 10 (= INR)
Difference between Von Willebrand and Haemophilia (in terms of PT, APTT and Bleeding Time)
Von Willebrand has increased bleeding time
Both have increased APTT (involves Factors 8 and 9)
Slight anaemia with hyperchromic cells lacking central pallor
Hereditary Spherocytosis
Warm vs Cold AIHA
Warm = IgG, which bind to RBCs at 37degrees
- Lymphoproliferative disorders
- Penicillin
- Autoimmune
Cold = IgM, which binds to RBCs at 4degrees
-Mycoplasma or EBV
Virus which causes Hodgkin’s
EBV
Causes primary effusion lymphoma
HHV8 (effusion in a tumour mass)
Neonate with thumb abnormalities and macrocytic anaemia (WBC and platelets normal)
Diamond-Blackfan Anaemia (affect RBC progenitors)
Condition increasing risk of thrombosis when on COCP
Factor V Leiden = activated Protein C resistance
When would you use cryoprecipitate?
Replace fibrinogen
-cryoprecipitate contains clotting factors and fibrinogen
What can CLL rapidly transform into?
Diffuse Large B cell non-Hodgkin’s lymphoma (=Richter’s transformation)
“Rain-drop skull” on X-Ray
Multiple Myeloma (multiple osteolytic lesions)
Clotting disorders associated with haemarthroses
Haemophilia A and B
Polycythaemia vera can transform to which leukaemia?
AML (>20% blasts on blood film)
Primary Myelofibrosis is associated with this genetic mutation
JAK2
Sickle Solubility Test is used to diagnose this
Sickle Cell Trait
Gene affected in Burkitt’s
c-myc
What is HbA?
a2b2 = 95% adult Hb
What is HbA2?
a2d2 = increased in beta thalassaemia as can’t make HbA
What is HbF?
a2g2 = foetal Hb
What is HbH?
b4 = in HbH Disease in alpha thalassaemia (3 alpha genes deleted)
What is Hb Barts?
g4 = alpha-thalassaemia major (4 alpha genes deleted)
What is HbS?
Hb found in sickle cell anaemia
Most common inherited bleeding disorder
Von Willebrand Disease
Pattern of inheritance in Von Willebrand
Autosomal dominant
How long should warfarin be used after DVT/PE?
3 months if provoked; 6 months if unprovoked
Rouleaux formation
Myeloma (stacking of RBCs)
Lymphoma which produces IgM
Waldenström’s macroglobulinemia
Proliferation of megakaryocytes –> bone marrow fibrosis
Myelofibrosis = tear drop poikilocytes, dry tap
Mechanism of febrile transfusion reaction
Recipient anti-HLA antibodies react against donor WBCs
Common lymphoid progenitor gives which 3 cells?
T, B and NK Cell (rest are from common myeloid)
Pneumonia bug causing autoimmune haemolytic anaemia
Mycoplasma (acute cold haemagluttinin)
IDA vs Anaemia of Chronic Disease
ACD: increased ferritin, reduced TIBC
VTE – how long warfarin and what INR target?
Warfarin for 3 months (6 if cancer)
INR 2.5-3.5
VTE while on warfarin - how long warfarin and what INR target?
Warfarin lifelong
INR 3.5
Emergency treatment in Haemophilia A vs B
A = cryoprecipitate (contains VIII) B = FFP
Hepatomegaly and chondrocalcinosis
Haemachromatosis
Vit B12 deficiency - loss of touch and proprioception, UMN signs, but pain and temperature spared
Subacute Combined Degeneration of Cord
Most sensitive antibody in pernicious anaemia
Anti-parietal cell
PML-PARA fusion gene
Acute Promyelocytic Leukaemia (t15;17)
Microcytic anaemia – reduced iron but increased ferritin
Anaemia of Chronic Disease (ferritin = acute phase protein)
Anaemia of Chronic Disease - what does liver produce which mediates the reduced iron?
Hepcidin (retains iron in macrophages and reduces export from enterocytes)
Immunoglobulin which binds to platelets in ITP
IgG
Apart from low VIII, what else is low in Von Willebrand?
Ristocetin
Hairy cell leukaemia express
Tartrate-resistant acid phosphatase
This degrades factor V
Protein C
Impaired degradation of factor Va and VIIIa
Protein S deficiency
Nuclei of cancer cells have cloverleaf apperance
Adult T-cell leukaemia
WBCs with filamentous projections/finger-like projections of cytoplasm
Hairy Cell Leukaemia
Blood film shows lots of RBCs stacked
Rouleaux = Multiple Myeloma
Hodgkin type that spreads contiguously
Hodgkin
Most common Hodgkin type
Nodular sclerosing (peaks in young women)
Treatment of Hodgkin relapse
High-dose salvage chemo –> autologous stem cell transplant
Waxing and waning lymphadenopathy but NO B symptoms
Follicular non-Hodgkin
First-line in myelodysplasia
Allogeneic Stem Cell Transplant
Which cells targeted in Aplastic Anaemia?
CD34 haematopoietic stem cells
Treatment of Aplastic Anaemia in
< 50yrs
> 50yrs
<50yrs: stem cell transplant
>50yrs: horse anti-thymocyte globulin and ciclosporin
Mode of inheritance of most common form of Dyskeratosis Congenita
X-linked (mutated DKC1)
Haematocrit target in polycythaemia vera
<45% (via venesection and hydroxycarbamide)
Most common cause of high platelets
Essential Thrombocythaemia = platelets > 600
Neutrophil precursor found in CML
Myelocytes
Targeted treatment for Philadelphia +ve leukaemia
Imatinib
Reverses warfarin in emergency
Prothrombin Complex Concentrate
Cause of Transfusion-Related Acute Lung Injury (TRALI)
Anti-WBC antibodies in donor –> clump with recipient’s WBCs
Jewish male presenting with multiple pathological fractures and hypersplenism
Gaucher’s disease
Which paraprotein is most commonly raised multiple myeloma?
IgG