Haematology Flashcards
Most common cause of basophilia
Myeloproliferative malignancy
Name 9 causes thrombocytopenia
Decreased production (HIDI)
- marrow hypoplasia: childhood bone marrow failure syndromes (Fanconi’s anaemia), drug induced (cytotoxics, antimetabolites), transfusion associated graft vs host disease
- marrow infiltration: leukaemia, myeloma, myel/fibrosis, lysosomal storage d/o eg Gaucher’s
- haematiniC deficiency: vitamin B12, folate deficiency
- familial thrombocytopathies: wiskott - Aldrich syndrome (small platelets)
Increased consumption IT.COM
- immune mechanism: ITP, post transfusion purpura, drugs (quinine, vancomycin, heparin )
- coagulation: DIC
- mechanical pooling: hypersplenism
- thrombotic microangiopathies: haemolytic uraemic syndrome (hus), liver disease, TTP, pre-eclampsia
- other: gestational thrombocytopenia, type 2b Von willebrand disease
Name 4 indications for bone marrow investigation
• Unexplained cytopenias
• suspected bone marrow infiltration by Tb or malignancy
• lymphoma staging
. Leukaemia follow up
Name 3 types microcytic (mcv < 80 ) anaemias and how to diagnose
DO serum iron studies
• iron deficiency anaemia. : low iron and ferritin (stores iron), high TIBC (total iron binding capacity)
• thalassaemia minor: Mentzer index MCV: RBC <13 ( abnormal hb)
• anemia of chronic disease component plus iron deficiency: low-normal iron, low-normal ferritin, low tibc
Name 7 types normocytic (mcv 80 -100 ) anaemias and how to diagnose
Do reticulocyte count.
<2% (hypoproliferative)
• leukaemias
• aplastic anaemia
• pure red cell aplasia
• other marrow failure syndromes: ineffective haematopaesis eg myelodysplastic syndrome
> 2% (hyperproliferative)
• haemorrhage
• haemolytic anaemias
- intracorpuscular defects : hereditary spherocytosis, thalassemia, sickle cell anaemia….
- extracorpuscular defects: incompatible transfusion, hypersplenism, trauma, dic…
Anaemia of chronic disease: chronic inflammation, ckD, malignancy, endocrine deficiency, liver disease, malnutrition
Name 7 types macrocytic (mcv > 100 ) anaemias and how to diagnose
Do blood smear and look at macrocytes
Megaloblastic oval macrocytes and segmented neutrophils
• vit b12 deficiency (pernicious anaemia, ileal disease, poor intake)
• Folate deficiency (dietary, alcoholics, coeliac disease, increased cell turnover, phenytoin, methotrexate, sulfasalazine )
Non-megaloblastic round macrocytes
• alcohol
• myelodysplastic syndrome, myeloproliferative disease
• liver disease: cirrhosis
• congenital bone marrow failure syndromes
• hypothyroidism, reticulocytosis (haemolysis, haemorrhage)
Name Virchow’s triad
Causes of venous thrombosis
• endothelial injury
• abnormal blood flow
• hypercoagulability
Which patients should have a thrombophilia screen (5)
Definitely:
• DvT /pe, intermediate risk recurrence
• vTE in unusual places eg peritoneal thrombosis, vessels supplying abdominal organs;unprovoked
Consider:
• arterial thrombosis in young, unexplained
• VTE patient requests testing
• family members if “strong thrombophilia” in index patient
Not unexplained pregnancy losses.
Name 5 indications for ordering haemostasis tests! Nb
• Identify which patients with acute bleeding have correctable bleeding tendency
. Identify which patients with acute clots have correctable clotting tendency
• monitor response to anticoagulant medication
• prognosis in liver failure
• screen for DIC
Name 5 conventional tests for haemostasis
• Platelet count: EDTA purple top
• pt/inr: citrate blue top
• aptt
• fibrinogen
• D dimer
Name 2 causes pseudothrombocytopenia
• Incomplete mixing of blood in collection tubes → formation of platelet trapping clots
. EDTA dependent agglutinins
Name 6 causes thrombocytosis
Reactive
• infection
• post surgery
• post splenectomy
• malignancy
• acute blood loss; haemolytic anemia
• acute + chronic inflammatory disorders; tissue damage
Clonal (primary/essential)
- Myeloproliferative disorders: CML
- polycythaemia rubra Vera
- myelOfibrosis
- primary thrombocythaemia
- myelodysplastic syndromes
Name the cause of an isolated prolonged pt
Factor VII deficiency
Name the cause of an shortened pt
Following treatment with rVll a
What is aptt?
Activated partial thromboplastin time
Time in seconds for fibrin cot to form
Test intrinsic (viii, ix, xi, xii ) and common ( ii,v, X , fibrinogen) path
Play Table Tennis INSIDE
Name 2 causes high fibrinogen
• Acute phase reactant
• pregnancy
Name 2 causes low fibrinogen
• Liver failure
• DIC
What does pt measure? (2)
• Extrinsic pathway: tf (tissue factor), FVII
• common pathway: ll,v,x, fibrinogen
What does aptt measure? (2)
• Intrinsic pathway: VII, ix, xi, xii
• common pathway: ll,v,x, fibrinogen
Name 4 causes isolated prolonged aptt
• Deficiencies viii, ix,xi, XIl (intrinsic pathway)
Contact factor deficiency
. Acquired clotting factor inhibitors
• lac
Name 7 causes prolonged aptt and pt
• Vitamin K deficiency
. Liver disease due to: malabsorption, decrease synthesis clothing factors, acquired dysfibrinogenaemia
• direct thrombin inhibitors
• DIC
. Afibrinogenaemia and dysfibrinogenaemia
. Dilution coagulopathy
• multiple clotting factor deficiencies
The following may or may not have prolonged pt: UFH, antiphospholipid antibodies, acquired clotting factor inhibitors
Platelets, INR, aptt, fibrinogen, D dimer in advanced liver disease?
Platelets low
, INR high
aptt normal to high
, fibrinogen low
, D dimer normal to high
Platelets, INR, aptt, fibrinogen, D dimer in DIC?
Platelets, low!
INR, normal to high
aptt, normal to high
fibrinogen, normal to low
D dimer high!
Platelets, INR, aptt (Activated partial thromboplastin time), fibrinogen, D dimer in haemophilia or antiphospholipid antibody syndrome?
Platelets, normal (coag defect)
INR, normal
aptt, high!
fibrinogen, normal
D dimer normal
Name 6 lab features of iron deficiency anaemia of peripheral blood and bone marrow
Peripheral
• hypochromic, microcytic red cells, target cells, pencil-shaped poikilocyles
• reticulocytopenia
• combined deficiencies or recent Iron therapy may → dimorphic blood film
• thrombocytosis
Bone marrow
• erythroblasts small with ragged cytoplasm
• absent iron stones and sideroblasts
Name 6 lab serum features of iron deficiency anaemia
• Reticulocyle haemoglobin content (chr) <27,2 (also in thalassaemia and hb-opathies)
. Iron falls, tibc rises
• ferritin related to tissue iron stones, decreased in iron and vitamin C deficiency
. S- transferrin receptor increased
• Red cell zinc protoporphyrin increased
• mentzer index (mcv/rcc) >13
What is the MCV / mch , serum iron, tibc, serum ferritin, bone marrow iron stores, erythroblast iron and hb electrophoresis in anaemia of chronic disease or malignancy
Hypochromic anaemia
MCV / mch , normal mild reduction
serum iron, reduced
tibc, reduced
serum ferritin, normal to high
bone marrow iron stores, present
erythroblast iron absent
and hb electrophoresis normal
What is the MCV / mch , serum iron, tibc, serum ferritin, bone marrow iron stores, erythroblast iron and hb electrophoresis, mentzer index and smear in thalassaemia
Micro cytic Hypochromic anaemia - low Hb!
MCV / mch , low
serum iron, normal
tibc, normal
serum ferritin, normal
bone marrow iron stores, present
erythroblast iron present (thus NOT fe deficient)
and hb electrophoresis, hba2 and HbF raised in beta form
mentzer index (mcv/rcc) <13 (this differentiates iron def vs thalassemia)
Normal porphyrin levels (fe def won’t)
Smear: micro cytic hypochromic, vary in size and shape, increased % reticulocyte, target cells, Heinz bodies
Name 5 broad causes iron deficiency anaemia with 2specific examples each
. Increased iron requirements: pregnancy, infants
• low intake: malnutrition, vegetarians
• decreased intestinal absorption: gluten induced enteropathy (investigate with endomysial and anti-transglutaminase antibodies, duodenal biopsy), pPI and H2B
• chronic blood loss: hookworm infections, NSAIDs, heavy menses, haematuria
• multiple causes: CKD (decreased absorption, blood loss, reduced hepcidin excretionand increased production, drugs, esas) , post op major surgery (blood loss, increase pro-inflamm cytokines)
Scientific name vitamin b 12?
Cobalamin
Name 5 causes vitamin b 12 deficiency
Severe deficiency
• severe malabsorption affecting physiological intrinsic factor cubam receptor axis: pernicious anaemia (autoimmune gastritis), gastrectomy, bariatric surgery, ileal resection
. Abuse nitrous oxide
• inherited metabolic: impaired ability to transport (tc deficiency),impaired ability to process
Mild to moderate
• mild to moderate malabsorption: gastritis, metformin, drugs that block stomach acid, chronic pancreatic disease
• dietary: vegetarians
Investigation if suspect pernicious anaemia clinically?
• Anti-intrinsic factor antibodies
(If negative, do serum gastrin. High in pernicious)
Name 8 clinical features folate deficiency anaemia
• Mild jaundice
• gradual onset with usual features of anaemia
• glossitis - beefy red sore tongue
• angular stomatitis
• purpura (thrombocytopenia)
• reversible melanin pigmentation
• progressive neuropathy due to b12 deficiency
• Risk neural tube defects and CVD (increase homocysteine)
Name 8 lab findings folate deficiency anaemia
• Oval macrocytes
• low reticulocyte count
• leucocyle count, platelets may be reduced
• hyper segmented neutrophils
• bone marrow: hypercellular (erythroid hyperplasia), megaloblasts, giant metamyclocytes and bands
• raised unconjugated bilirubin
• raised LDH
• raised homocysteine
Name 6 lab features haemolytic anaemia
• Normal or low hb
• biochemistry: high unconjugated bilirubin!, low haptoglobin, high f-sterCobilinogen and u-urobilinogen, high LDH!
• high reticulocyle count!
• blood film: polychromAsia, normoblasts, red cell shape changes! Eg schistocytes, spherocytes
• bone marrow: hyperplastic enythropaeisis
Name 8 causes intravascular haemolysis
• Mismatched blood transfusions,
. G6PD deficiency with oxidant stress
• Red cell fragmentation syndromes
• some auto-immune haemolytic anaemias
• drug and infection induced, haemolytic anaemias
• paroxysmal nocturnal haemoglobinuria
• March haemoglobinaria
• unstable haemoglobin
Name 9 causes haemolytic anaemia
Hereditary
• rc membrane abnormality: hereditary spherocytosis , elliptocytosis
• rc enzyme deficiency: G6PD deficiency,
• haemoglobin: deficiency eg thalassaemia; abnormality eg sickle cell disease
Acquired
- immune
→ autoantibodies
> warm antibodies (80%): primary idiopathic or secondary (autoimmune eg sle,ra; drugs eg L-dopa, methyldopa, penicillin ; lymphoid malignancy eg leukemia, lymphoma; other malignancy eg lung, colon, kidney, ovary, thymoma; other eg uc, HIV) (infection
> cold antibodies: primary idiopathic, secondary ( infection eg mycoplasma pneumonia, EBV, syphilis; lymphoproliferative disorders eg lymphoma)
→ alloantibodies:
> red cell antigen induced: transfusion reaction, haemolytic disease of newborn
- non immune
→ mechanical: prosthetic valve, microangiopathic eg DIC HUS TTP, March haemoglobinuria
→ infection: malaria, C perfringers toxin
→ chemical/physical: oxidative drugs eg dapsone, Wilson’s disease, burns, drowning
→ acquired abnormal membrane: paroxysmal nocturnal haemoglobinuria
Treatment hereditary spherocytosis? (4)
• Folic acid prophylaxis for life
• Blood transfusion if severe
• splenectomy
• cholecystectomy (gall stones 50% )
Treatment (beta) thalassaemia? (5)
• Maintain hb > 9-10
•Iron chelation therapy ( iron overload)
• immunise: hep B
• splenectomy (splenomegaly)
• bone marrow transplant (hypercellular bm - erythroid hyperplasia)
Treatment warm type autoimmune haemolytic anaemia (7)
• Remove underlying cause eg drugs (fludarabine, methyldopa)
• corticosteroids 1-2 mg/kg/day
• splenectomy (splenomegaly)
• rituximab
. Cytotoxic immunosuppressive drugs: azathioprine, cyclophosphamide, cyclosporine
• iv ig
• blood transfusion
Treatment cold type autoimmune haemolytic anaemia (7)
• Management difficult. Avoid cold exposure
. Folic and
• rituximab
• alkylating agents: chlorambucil, cyclophosphamide
• blood transfusion and plasma exchange
(Corticosteroids and splenectomy don’t work like in warm type)
Diagnosis paroxysmal nocturnal haemoglobinuria? (3)
• Positive acid lysis (ham) test
• absence Cd 55 and CD59 antigens
• FLAER (fluorescin labelled pro- aerolysin)
Name the blood donation donor safety checklist (10)
• Voluntary
• age 17 -70 (max 60 first donation)
• weight > 50 kg
• hb >13 male, > 12 female
• minimum donation interval 12 weeks, 6 weeks recommended (3x/year)
• pregnancy excluded
• medical exclusion criteria eg diabetes, cardiac disease, some git conditions
• vaccinations eg MMR - defer for 2 months
• acupuncture, body piercing - defer for 6 months
• test blood group, rh, HIV 1 2, HBV and HCV, syphilis
Name the 4 laws /legal aspects that govern blood transfusion
• National Health act 2003
-informed consent will avoid all liability!
-delictual liability (transfusion reaction)
-criminal liability (death)
Name 4 indications red cell concentrate
What is it: red cells in solution of glucose + adenine in saline.
• Acute blood loss!
• surgery
• anaemia: symptomatic severe!
• obstetric haemorrhage
Name 4 indications platelet transfusion,
• thrombocytopenia eg in acute leukaemia!
• Bone marrow failure
• massive transfusion
• acute DIC
• congenital disorders of platelets
Name 3 contra indications platelet transfusion
• ITP, unless bleeding
• TTP thrombotic thrombocytopenia purpura
• HIT heparin induced thrombocytopenia
Name 5 plasma products
• FFP
• cryoprecipitate
• coagulation factor concentrates
• human albumin
• immunoglobulins
Name 6 indications fresh frozen plasma
• Replacement inherited single factor deficiencies if single factor unavailable!
• multiple coagulation deficiency!
• TTP!
• Reversal of warfarin if active bleed
• vit K deficiency
• haemolytic disease of the newborn HDN
Give with RCC to avoid dilution coagulopathy
Name 3 indications coagulation factor concentrate
• Haemophilia
• haemo solvate factor viii
. Haemosolvex factor ix
Name 7 early complications blood transfusion
• Haemolytic reactions acute (will have fever) and delayed
• bacterial contamination. (fever)
. Anaphylactic reaction
• febrile non haemolytic transfusion reaction (fever)
• TRALI (fever)
•Allergic reaction
• electrolyte imbalances
Name 4 late complications blood transfusion
• Transfusion associated graft vs host disease (will have fever )
• transmission of infection (will have fever)
• post transfusion purpura
• alloimmunisation
Delayed haemolytic reaction (fever), iron overload
Management of reaction to blood transfusion? (6)
• Stop transfusion and administer iv fluids
• repeat ID and verification patient and unit
• specimens: admin set, unit of blood, posttransfusion patient sample (one clotted, one anticoagulated), urine sample (if suspect haemolytic reaction)
• Lab: repeat Abo and Rh of pre transfusion and post, DAT, crossmatch using pre and post samples, check for huemolysis
• Blood cultures, haptoglobin, FBC, urine haemosiderin
• complete reaction report form
Blood drawing order? (7)
Blue citrate
Red serum
Yellow SST
Green heparin
Purple EDTA
Light purple pink cross match
Grey fluoride oxalate
Name 4 causes neutropenia
Decreased production
• General bone marrow failure: aplastic anaemia, acute anaemia, MDs, chemo
• specific neutrophil dysfunction: congenital, drug induced
Increased destruction
• General: hypersplenism
• specific: immune mediated destruction
• benign ethnic neutropenia Jewish, black
Name 5 causes neutrophil leucocytosis
• Bacterial infection = leukaemoid reaction!
• pregnancy
• tissue necrosis
• myeloproliferative neoplasms and other neoplasms
• acute haemorrhage or haemolysis
• drugs - corticosteroids
• g-csf (myeloid growth factor)
• asplenia
Name 6 causes monocytosis
• Chronic bac infections eg Tb
• connective tissue diseases eg SLE
• protozoan infections
• chronic neutropenia
• Hodgkin lymphoma, AML and other
. Chronic myelomonocytic leukemia
Name I0 causes eosinophilia
• Allergy
• parasitic diseases
• recover from acute infection
• certain skin infections eg psoriasis
• polyarteritis nodosa, vasculitis, serum sickness
• drug sensitive
• G vs H disease
• Hodgkin disease
• metastatic malignancy with tumour necrosis
• hypereosinophilic syndrome
• pulmonary syndromes
• chronic eosinophilia leukaemia, myeloproliferative
Name 2 causes lymphocytosis
. Infections mostly viral
. Lymphoproliferative malignancy
Name 5 causes lymphopenia
• Immune deficiency syndrome
. Irradiation
• acute stress ie trauma surgery
• drugs - corticosteroids
• autoimmune disease
Name 4 indications bone marrow aspirate and trephine
- Unexplained cytopenia
- suspected bm infiltration by Tb or malignancy
- lymphoma staging
- leukemia follow ups
Diagnosis VTE? (4)
- Clinical suspicion: wells score
- d dimer
- compression ultrasound
- other: contrast venography, MRI
Diagnosis pulmonary embolism (7)
- Clinical: wells score
- CXR, ECG
- D dimer
- v/q
- Ct or MRI angiography
- pulmonary angiography
Define heparin induced thrombocytopenia (hit)
- Heparin joins platelet factor 4 to form complex
- antibodies against complex → platelet activation and platelet thrombi formation
- platelet consumption → thrombocytopenia
Stop heparin, find alternative
Name 8 causes high D dimer
- Vte
- arterial clot
- DIC
- Severe sepsis
- malignancy
- Liver disease
- recent surgery, trauma
- pregnancy
Basically: recent intravascular coagulation
What is serum iron?
Ferric (fe 3+) ions bound to serum transferrin
Poor marker of iron status
What is ferritin and what does it mean if it’s increased/decreased?
Intracellular iron storage protein. Indicate body’s total iron stores
Low = iron deficiency! (Most specific marker)
High = iron overload or non-specific for alcohol, liver disease, chronic inflammation
What is transferrin?
Transport protein that binds to Iron in plasma
High - iron deficiency
Low - chronic disease, acute phase reaction, iron overload
What is total iron binding capacity?
The total amount of iron that can be bound to serum transferrin.
Thus directly proportional to transferrin
High - iron deficiency
Low - chronic disease, acute phase reaction, iron overload
Indication cryoprecipitate? (2)
What is it: fibrinogen + factor viii + Von willebrand factor
- Von willebrand disease
- haemophilic
