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158 Cards in this Set

  • Front
  • Back

What is the life cycle of an erythrocyte?

Erytroblast to Reticulocyte and then erythrocyte

What stimulates erythropoiesis?

Erythropoeitin

Where is it made and why?

In the kidneys in response to hypoxia

What is reticulocyte count a measure of?

Red cell production

What regulates production of platelets?

Thrombopoietin

Where is it produced?

The liver

What do neutrophils do?

Ingest and destroy bacteria and fungi

What do increased neutrophils show?

Infection or inflammation

Decreased neutrophils?

Marrow failure


Sepsis


Autoimmune disease

What do monocytes do?

Ingest and destroy bacteria and fungi

What are monocytes known as in tissues?

Macrophages

When are eosinophils increased?

In allergic reactions and parasitic infection

What types of lymphocytes are there?

B, T & NK

Where are B and T cells produced and where do they mature?

B - bone marrow for both


T - produced in bone marrow, mature in thymus

What can cause splenomegaly?

Infections - EBV, TB


Malignancies - Leukaemia


Portal Hypertension


Haemolytic disorders - hereditary spherocytosis


Connective tissue disorders - SLE

What is anaemia?

Reduction in red cells or their Hb count

What materials are essential for red cell production?

Iron


B12


Folic acid


Erythropoietin

What is hereditary spherocytosis?

Defects in red cell membrane proteins causes cells to be spherical

How does it present?

Anaemia


Jaundice


Splenomegaly


Pigment gallstones

Management?

Folic acid


Transfusion


Splenectomy

What does G6PD do?

Prevents red cells from oxidative damage

How is G6PD deficiency passed on?

X-linked

What is seen on blood film?

Blister cells

How does it present?

Neonatal jaundice and anaemia


Often precipitated by infection or eating broad beans

How is sickle cell caused?

Beta gene is replaced with sickle gene

What happens?

Causes the production of sickle cells, which causes increased haemolysis which leads to vaso-occlusion

How does it present?

Sickle cell crises - bones and chest commonly affected


Increased infection risk


Anaemia

What is the life long management of sickle cell?

Vaccinations


Penicillin prophylaxis


Folic acid


Blood transfusions


Hydroxycarbamide


Bone marrow transplant

What is the management of a sickle cell crisis?

Hydration


Oxygen


Quick treatment


Analgesia - opiates

What causes thalassaemias?

Reduced or absent globin chain production due to mutation or deletion in gene

Which type is incompatible with life?

Alpha thalassaemia

What does beta thalassaemia major present like?

Severe anaemia at 3-6 months


Expansion of bone marrow


Splenomegaly


Growth retardation

Treatment?

Chronic transfusion support with deferasirox as iron chelation therapy


BMT is curative

Explain the RhD blood groupings

RhD negative people can only receive RhD negative blood, but RhD positive can receive any.

Who is given anti-D as an injection to prevent rhesus disease?

Pregnant RhD negative women

What is donor blood screened for?

Hep B


Hep C


HIV


Syphilis

What is readily available in case of emergency?

O- red cells and AB plasma

What is the massive haemorrhage policy?

Immediate supply of:


6 units of red cells


4 units of FFP


1 units of platelets



What is a minor reaction to a blood transfusion?

Fever and hives

Major reaction?

Fever


Hives


Respiratory distress


Hypotension


↑HR

What is the normal range of Hb in males?

12-70 (140-180)


>70 (116-156)

In females?

12-70 (120-160)


>70 (108-143)

What are the clinical features of anaemia?

Tiredness


Breathlessness


Ankle oedema


Dizziness


Chest pain


Factors relating to underlying cause

What tests should be done?

MCH and MCV

Types of microcytic anaemia?

Iron-deficiency anaemia


Thalassaemia


Sideroblastic anaemia

Causes of IDA?

Blood loss


Poor diet


Malabsorption

What signs may be present in IDA?

Koilonychia


Atrophic glossitis


Angular cheilosis

What test is used to confirm diagnosis?

Serum ferritin - will be reduced

Treatment?

Treat the cause


Give iron tablets

Causes of normocytic anaemia?

Acute blood loss


Anaemia of chronic disease


Bone marrow failure


Renal failure


Hypothyroidism

Causes of macrocytic anaemia?

B12 or folate deficiency


Alcohol excess


Myelodysplastic syndromes

Where is iron stored in the plasma?

Transferrin in plasma


Ferritin in macrophages and hepatocytes

What does hepcidin do?

Reduces intestinal iron absorption and iron mobilisation

When is hepcidin produced?

In response to inflammation so can cause anaemia (anaemia of chronic disease)

What does B12/folate deficiency show on blood film?

Megaloblastic anemia

What tests should be done?

Blood film


Bone marrow biopsy possible

What are causes of B12 deficiency?

Pernicious anaemia


Gastric/ileal disease

What is pernicious anaemia?

Antibodies are produced against intrinsic factor and gastric parietal cells

Treatment?

B12 IM injections

Causes of folate deficiency?

Diet


Increased requirement


GI pathology

Treatment?

Oral folate replacement

What is used to form a clot?

Platelets


Von willebrand factor


Coagulation factors

What activates the formation of a clot?

Abnormal surfaces and physiological activators

How is the clot formed?

Platelets adhere to the collagen and form a plug. A fibrin meshwork forms over the top

What is the cause of haemophilia A & B?

A - Absent factor VIII


B - Absent factor IX



How does the clot remain confined to the site of the injury?

Due to TFPI, Protein C & S and antithrombin which are natural anticoagulants

What gets rid of clot after it is needed?

Fibrinolytic system

How does it work?

t-PA and u-PA activate plasminogen to form plasmin which breaks down fibrin clot.

What marker is released as part of this process?

D-dimer

What is a thrombus?

A clot arising in the wrong place

What is a thromboembolism?

The movement of a clot along a vessel

What is an arterial thrombus made up of?

Platelets and fibrin

What is it secondary to?

Atherosclerosis

What examples?

Coronary - MI, angina


Cerebrovascular - Stroke, TIA

RFs for Art thrombus?

Age


Smoking


HT


Diabetes

What is used to treat?

Thrombolysis and antiplatelets

What is a venous thrombus made up of?

Fibrin and red cells

What are the causes of VT?

Stasis


Hypercoaguability


Vessel damage

What examples?

DVT


PE

Risk factors for VT?

Age


Pregnancy


Surgery


Immobility

What are used as probability scores for VT?

Wells


Geneva

What marker is elevated in VT?

D-dimer

What tests can be done?

Doppler US


V/Q scan

What is used to treat?

LMW Heparin

What are the causes of DIC?

Septicaemia


Malignancy


Eclampsia

What is the platelet pattern of bleeding?

Mucosal


Epistaxis


Purpura


Menorrhagia


GI bleeding

What are purpura?

Red/purple spots that do not blanch under pressure

What is coagulation factor pattern of bleeding?

Articular


Muscle haematoma


CNS


retro-peritoneal bleeding


Post-surgical bleeding

What is the inheritance of Haemophilia?

X-linked

Which type is more common?

A

Presentation?

Coagulation Factor type bleeding

Complications?

Synovitis


Chronic arthropathy


Neurovascular compression

Diagnosis?

Prolonged APTT


Normal prothrombin time and bleeding time


Reduced factor 8/9

Treatment?

Factor 8 or 9 replacement


DDAVP is given for A


Tranexamic acid

What is the inheritance of VWD?

Autosomal dominant

Presentation?

Platelet type bleeding

Diagnosis?

Increased APTT


Increased bleeding time


↓VwF



What should not be given to patients with VwF disease?

NSAIDs

Treatment?

vWF concentrate or DDAVO


Tranexamic acid

What is the presentation of thrombocytopenia?

Mucosal/Platelet type bleeding with petechiae and ecchymosis

Tell me about acute leukaemia?

Leukaemic cells do not differentiate


Bone marrow failure


Rapidly fatal if untreated


Potentially curable

Chronic leukaemia?

Cells retain ability to differentiate


Proliferation without Bone marrow failure


Survival for a few years


Not curable without BMT

Presentation of AML?

Bone marrow failure


Hepatomegaly


Splenomegaly

How does BMF present?

Anaemia


Platelet type bleeding


Susceptible to infection

Investigations?

Bone marrow biopsy


Immunophenotyping done to differentiate from ALL

What is seen on microscopy in AML?

Auer rods

Treatment?

Supportive Care - Blood transfusions, IV fluids, Allopurinol


Chemotherapy - Intensive


BMT

Who gets CML?

People between 40-60

What genetic component is present in CML?

Philadelphia chromosome

Symptoms of CML

Chronic and insidious


Systemic - weight loss, tiredness, fever, sweats and gout


Platelet type bleeding

How does gout occur?

Due to purine breakdown

Signs of CML?

Splenomegaly


Hepatomegaly


Anaemia


Bruising

Diagnosis

↑↑WBC


Blood film shows all stages of white cell differentiation


Bone marrow and blood cells contain PH

Treatment

Imatinib - Tyrosine kinase inhibitor


Stem cell transplantation is an option

What is an example of a myelodysplastic syndrome?

Polycythaemia Vera

What is polycythaemia vera?

Malignant proliferation of a clone derived from one pluripotent stem cell which leads to excess porliferation of RBCs, WBCs and platelets

What does this lead to?

Hyperviscosity


Thrombosis

Presentation?

Itch after a hot bath


Erythromelalgia - burning sensation in toes and fingers


Headaches, dizziness, tinnitus and visual disturbance are possible

Signs?

Facial flushing


Splenomegaly

Investigations?

↑Hb concentration


↑Haematocrit

What mutation is present?

JAK2

Treatment?

Keep haematocrit below 0.45 (Prevent thrombosis)


Venesection in younger patients


Hydroxycarbamide

Complications?

Can progress to AML

What is the most common cancer of children?

Acute Lymphoblastic Leukaemia

What is it?

Neoplastic disorder of lymphoblasts which mostly affects B-cells

Presentation?

2-3 history of bone marrow failure or bone/joint pain

Signs?

Splenomegaly


Hepatomegaly


Lymphadenopathy


Weight Loss

Diagnosis?

>20% lymphoblasts present in bone marrow


High WCC

What is the treatment?

Supportive - blood transfusions, IV fluids, allopurinol


Antibiotic prophylaxis


Chemotherapy


Stem cell transplantation

What is Chronic Lympocytic Leukaemia?

Accumulation of mature B cells that have escaped programmed cell death and undergone cell-cycle arrest in the G0/G1 phase

Presentation?

Often asymptomatic


Anaemic or prone to infection



Signs

Lymphadenopathy


Splenomegaly

How does it often present?

As a surprise finding on a routine FBC

How is diagnosed

Increased number of lymphocytes on blood film

When is treatment indicated?

Only if symptomatic, progressive BMF or massive lymphadenopathy

What is the treatment?

Fludarabine


Cyclophosphamide


Rituximab

What is lymphoma?

Malignant proliferation of lymphocytes

What happens?

The lymphocytes acccumulate in lymph nodes causing lymphadenopathy.

What is the difference between Hodgkins and Non-Hodgkins?

Reed-sternberg cells are present in hodgkins lymphoma

Presentation?

Lymphadenopathy


Hepatosplenomegaly


Extranodal disease


B-symptoms - fever, night sweats and weight loss


Bone marrow involvement

How is it staged?

Lymph node biopsy

What age gets hodgkins?

Bimodal age curve


1st peak 15-35


2nd peak in elderly

What infection is associated with HL?

EBV infection

Treatment

Combination chemotherapy

How many subtypes of NHL are there?

50 subtypes

What type of cell are they most derived from?

B-cells

How does low-grade present?

Often Asymptomatic


Responds to chemo


Incurable

How does high grade present?

Often aggressive


Requires combination chemo


Can be cured

What is the most common High grade?

Diffuse large B-cell lymphoma

Low grade?

Follicular lymphoma

What is myeloma?

Neoplastic disorder of plasma cells which results in excessive production of an immunoglobulin

What immunoglobulins are most common?

IgG and IgA

Presentation?

Bone lesions


Bone marrow failure


Renal Failure


Prone to infections

What do the bone lesions cause?

Pathological fractures


Backache


Vertebral collapse


Hypercalcaemia

Investigations

Monoclonal protein in serum or urine electrophoresis


Increased plasma cells on marrow biopsy


X-ray reveals bone lesions

Treatment

Chemo


Bisphosphonates


Blood transfusion


Surgery

What is a complication of any haematological malignancy?

Infection

What are measures taken to prevent this?

Antimicrobial prophylaxis


Stem cell transplant


Vaccinations


IV Immunoglobulin replacement