Blood Disorders Flashcards
A patient’s blood results show high Eosinophils. What could that indicate?
Allergic reaction or Asthma
(=Eosinophils participate in the inflammatory response of these 2 scenarios)
A patient’s blood results show high Neutrophils. What could that indicate?
1.Infections usually due to bacterial / Fungi / Parasitic causes.
2.Corticosteroids can also cause an increase
A patient’s blood results show high Basophils. What could that indicate?
1.Peristant Infections such as Influenza or TB
2.Allergies
3.Could be more serious: Hyperthyroidism or Cancer
If a patient is smoking, what happens to the RBC levels and why?
INCREASE:
Carbon monoxide from the cigarettes has a higher affinity for haemoglobin than O2 therefore there is less bound O2. Less O2 levels triggers the production of Erythropoietin from the kidneys which increases RBC production.
If there are high Basophils, what effects would you expect in terms of blood clotting?
Decrease in clot formation as Basophils release Heparin which prevents clotting.
Why can’t a pregnant woman towards the end of
her pregnancy not be able to take Aspirin?
Aspirin inhibits prostaglandin synthesis which therefore inhibits thromboxane synthesis. Thromboxane is required for platelet activation and vascular spasm (closing of blood vessels) and so by inhibiting this, during labour, less clotting would mean EXCESSIVE bleeding putting both Mum and Baby at risk.
A patient has a suspect heart attack. What medication would usually be given on route to the hospital in the ambulance and why?
Aspirin
=to reduce clotting that has most likely caused a heart block
What is gold star treatment of clots in hospitals?
Streptokinase
What is the pathophysiology of Haemolytic Disease of the Newborn?
When a RH- mother has a baby that is RH+. The mother produces antibodies to destroy the baby’s RBCs. This is mainly common in second, third etc pregnancies since the first pregnancy would not be enough time for antibody production. Baby’s life is therefore at risk or if born will need to treatment to reverse these issues.
A baby is born with Hemolytic Disease. What are the treatments?
1.Phototherapy
=to treat the Jaundice (Bilirubin from destruction of their RBCs)
2.Exchange transfusion (RH- blood)
3. Erythropoietin
=(treats the anaemia)
What are the causes of Secondary Erythrocytosis?
DECREASED O2 LEVELS by:
-High Altitudes
-Chronic Obstructive Pulmonary Disease (COPD)
-Congestive Heart Failure
What is Erythocytosis?
An overabundance of RBCs increasing blood viscosity which causes reduced blood flow and severely plugged capillaries.
What is Primary Erythrocytosis?
Also known as Polycythemia Vera.
-Increased RBCs and decreased blood flow
How is Erythrocytosis treated?
Phlebotomy - removal of RBCs
An athlete has been blood doping. What is a serious complication of this?
Erythocytosis
Name some symptoms of Erythrocytosis?
Headaches.
blurred vision.
red skin, particularly in the face, hands and feet – this may be more difficult to see on black or brown skin.
tiredness.
high blood pressure.
dizziness.
discomfort in the tummy.
confusion.
What is the Haematocrit measurement?
The percentage of blood volume that is RBCs.
What are the causes of Aplastic Anaemia?
Remember mneumonic
CARB mneumonic
-Chloramphenicol (Broad-spectrum Antibiotic)
-Autoimmune destruction of red marrow stem cells (therefore less production of RBCs)
-Radiation / Chemotherapy
-Benzene exposure e.g Car Exhaust / Cleaning products, Paint, Glue
What is the most common type of anaemia for a smoker?
Iron-deficiency anaemia (due to the carbon monoxide binding the haemoglobin instead of O2)
What are the symptoms of aplastic anaemia?
Usual anaemia symptoms - pale and tired
+Frequent infections
+Dyspnoea during exertion
Aplastic anaemia is less RBCs due to less production which means less O2 transport and as RBCs acts as biochemical barriers to infection, less RBCs means more infection.
A high MCV of 80-100 would indicate which pathologies?
Haemolytic anaemia and aplastic anaemia
What blood test results would be expected from a patient with aplastic anaemia?
HIGH MCV and Low Reticulocytes (immature RBCs)
What does MCV measure?
Size of your RBCs
What blood results are expected for iron deficiency anaemia?
LOW MCV and MCHC
A pregnant woman has suspected Folate deficiency anaemia. What would you expect the MCV to be like and what would you do next?
-MCV could be either high or normal.
-Cobalamin test is required even if MCV is normal to test for B12 and therefore the potential neurological impacts (e.g Spina Bifida)
What is Colabamin testing?
Testing for B12
What is produced to facilitate B12 intestinal absorption and where is it made?
Intrinsic Factor from the stomach
What is the pathophysiology of Pernicious anaemia?
B12 deficiency which is usually caused by an autoimmune response destroying the cells of the stomach that produce intrinsic factor.
What are the common causes of an increased Prothrombim time?
Remember pneumonic
Don’t Lengthen Afghanistan’s War
1.Deficiency (of Vit K)
2.Liver disease
3. ALL Antibiotics
4. Warafin
What can decrease Prothrombin time?
Vitamin K, Oral contraceptive pill
What would you expect of the MCV for a patient with iron deficiency anaemia?
Low - i.e Microcytic
Name common types of microcytic anaemia.
1.Iron deficiency anaemia
2.Thalassaemia
3.Sideroblastic anaemia (but can also be normocytic)
Name common causes of macrocytic anaemia.
Remember mneumonic
Many Parties Allow Copious Alcohol
1.Methotrexate
2.Pernicious Anaemia
3.Anti-convulsant
4.Cancer (Myelodysplastic)
3.Alcohol
What group of people are at risk of iron deficiency anaemia?
Pre-menopausal anaemia (due to increased menstrual bleeding)
What is the most common cause of anaemia in men and post-menopausal women?
GI bleeding
Name 2 clinical signs of iron deficiency anaemia?
Koilonychia (spoon shaped nails)
-Angular chelitis (thickening of skin around lips at the side)
A patient with suspected anaemia has chest pains and shortness of breath. Could this still be anaemia?
Yes, but these symptoms only develop in late and progressed anaemia
What is another name for Plummer-Vinson Syndrome?
Paterson-Kelly-Brown Sydrome
What are the classical triad of symptoms for a patient with Paterson-Kelly Brown Syndrome?
1.Iron Deficiency Anaemia
2.Oesophageal Webs (thin membranes within the upper oesophagus causing difficulty swallowing)
3.Dysphagia
What is the treatment for Plummer-Vinson Syndrome?
-Iron replacement
-Endoscopic dilatation
+Monitoring for Oesophageal cancers
What is diagnostic of iron deficiency anaemia?
Low Serum Ferritin
When can serum ferritin be falsely elevated?
During an inflammatory illness - this would raised
What is the stepwise management for iron deficiency anaemia?
1.Oral Iron Replacement:
-Ferrous Sulphate / Fumarate
A patient is put on iron replacement. What should they be warned of?
-Black stools
-Constipation
-Nausea
What is the treatment for Thalassaemia?
Blood transfusions
+Iron Chelation (to reduce iron overload from the transfusion)
=Desferrioxamine (subcutaneous)
What is used to treat congenital Sideroblastic Anaemia?
Pyridoxine
B6vitamin that will supports proper development of RBCs as it is important for Haem Synthesis. Works within 2 weeks.
What is Sideroblastic Anaemia?
Dysfunctional Erythropoiesis leading to excess iron deposition
What are the causes of acquired Sideroblastic Anaemia?
Remember mneumonic
Sideroblastic Anaemia Angers Life
-Systemic Lupus Erythematosus
-Alcohol
-Antibiotics
-Lead poisoning
What are the 3 causes of B12 insufficiency ?
1.Dietary e.g Vegetarians and Vegans
2.Gastric Pathology (e.g pernicious anaemia)
3.Small bowel pathology (Inflammatory Bowel Disease, Malabsorption disorders e.g Small Intestine Bacterial Overgrowth)
What are the causes of folate deficiency?
Remember mneumonic
Men Deficient in Proper Manners Might Tremble
1.Metabolic demand increased (pregnancy, proliferative diseases, malignancy)
2.Diet (alcohol and malnutrition)
3.Phenytoin (drug for seizures)
4.Methotrexate
5.Malabsorption disorders (e.g Irritable Bowel Disease, Small Intestinal Bacterial Overgrowth)
6.Trimethoprim (drug for UTIs)
Name 3 common iron chelators and what do they do?
=To prevent iron overload in patients getting regular blood transfusions
1.Deferasirox (oral)
2.Deferiprone (oral)
3.Desferrioxamine (subcutanous)
All start with Des/Def -
The Ox is Prone to Amines
What are the symptoms of a folate deficiency?
Usually asymptomatic but can present with
GLOSSITIS
=Inflammation of the tongue
What is the pathophysiology of Pernicious Anaemia?
Atrophic gastritis and destruction of parietal cells (that produce intrinsic factor)
NOTE: Intrinsic factor is required for B12 reabsorption so this causes a deficiency of B12
What is the average onset for Pernicious Anaemia?
60 and above
What autoimmune conditions can secondarily cause Pernicious Anaemia?
-Addison’s disease
-Vitiligo
-Hashimoto’s Thyroiditis
-Grave’s Disease
Think ENDOCRINE!
A patient has Pernicious Anaemia. What investigation is required to monitor for what potential serious complication?
Upper GI endoscopy for Gastric Tumour monitoring
What is the management for B12 deficiency?
6 doses over 2 weeks of IM B12 injections
What are the 5 sickle cells crises / presentation (if not diagnosed at birth)?
1.Vaso-Occlusive Crisis
=skeletal pain due to necosis of bone marrow - fingers is common for children (Dactylitis) and torso bone pain for adults
2.Blood vessel crisis e.g Bowel ischaemia, renal papillary necrosis, retinal haemorrhage / detachment, cerebral infarction
3.Sequestration crisis
=Splenomegaly, Pallor
4.Acute chest syndrome
=Vaso-occlusion of the lungs (seen on CXR) presenting chest pain, fever, dyspnoea
3.Aplastic Crisis
=Severe anaemia causing a similar clinical picture to heart failure
What is given to patients with sickle cell anaemia?
Hydroxyurea (increases concentration of Hb)
-bone marrow replacement in children
What is the treatment for Aplastic Anaemia for young patients (under 50s) and Elderly (over 50)?
Under 50s:
-Allogenic Stem Cell treatment
–>curative
Over:
-Immunotherapy (Cyclosporin and Antithymocyte globulin)
–>improves survival
For remembering Immunotherapies:
-Cylone Spores
-Aunty Thyme -ocyte
What is the main major complication of Hereditary Spherocytosis?
Gallstones
=The red cell lysis causes an accumulation of unconjugated bilirubin which can therefore cause Gallstones
What is the line of inheritance for Hereditary Spherocytosis?
Autosomal dominant
What is Hereditary Spherocytosis?
Inherited haemolytic anaemia where defects in protein synthesis increase RBC fragility and therefore haemolysis develops.
How is Hereditary Spherocytosis diagnosed?
Peripheral blood film - presence of Spherocytes.
What is the presentation of a patient with Hereditary Spherocytosis (incl onset)?
Present at birth:
-Jaundiced
-Splenomegaly (most not all)
-Megaloblastic crisis (folate deficiency)
-Aplastic crisis due to B19 Parvovirus
Megaloblastic crisis
What haematological crisis can a B19 Parovirus cause (usually in children)?
Aplastic Anaemia Crisis
What is the treatment for Hereditary Spherocytosis?
1.Folate replacement and splenectomy AFTER THE AGE OF 6
A 3year old is being considered for a Splenectomy. What should be considered and why?
Operation not until 6years old due to risk of SEPSIS.
What is given post-splenectomy and for how long?
For at least 2 yrs after operation.
Vaccines:
-Pneumococcal vaccine
-Meningococcal ACWY conjugate vaccine -Meningococcal B vaccine
Antibiotics:
Amoxycillin or Clarithromycin
-Patients should always have access to these to take immediately after signs of an infection
What is the inheritance for Glucose-6-phosphate dehydrogenase (G6PD) deficiency.
X linked
(more common in men)
What is the pathology of Glucose-6-phosphate dehydrogenase (G6PD) deficiency?
Deficiency of G6PD means there is an intolerance of oxidative stress and therefore increasing haemolysis.
–>Haemolysis is triggered by drugs, acute infection and exposure to certain substances.
In Glucose-6-phosphate dehydrogenase (G6PD) deficiency, what are the triggers for haemolysis?
Drugs:
-Antimalarials (Chloroquine phosphate).
-Aspirin
-Antibiotics
-Vitamin K
Acute Infection
Exposure:
-Broad / Fava Beans
-Moth balls
What is the presentation of a patient with Glucose-6-phosphate dehydrogenase (G6PD) deficiency?
Jaundice and anaemia
A patient with Glucose-6-phosphate dehydrogenase (G6PD) deficiency has a blood film come back after a suspected acute attack. What would you expect to see?
Blister (/bite) cells and Heinz bodies
-may also see elevated reticulocyte count
A patient has suspected G6DP. What tests should be done / considered?
-Blood film
(look for Blister cells and Heinz bodies, elevated reticulocyte count)
What is the management of Glucose-6-phosphate dehydrogenase (G6PD) deficiency?
Acute transfusions in SEVERE cases
-stop triggers and treat underlying cause (acute infection)
What is inheritance of Pyruvate Kinase Deficiency?
Autosomal Recessive
What is the pathology of Pyruvate Kinase Deficiency?
Embden-Meyerhof glycolytic pathway is affected causing reduced ATP synthesis which causes red cells to be RIGID.
A patient has suspected Pyruvate Kinase Deficiency, what investigation should be done and what result would you expect?
Blood film
=Prickle cells
What is the presentation of a patient with Pyruvate Kinase Deficiency?
Variable of splenomegaly and anaemia.
What is the management for Pyruvate Kinase Deficiency?
Possible transfusions and splenectomy if the patients needs regular transfusions
What is the Coombs Test and what does it diagnose?
An antiglobulin test to detect presence of antibodies.
- DAT coombs is for testing for Haemolytic anaemia and Transfusion reactions, while
-IAT is for prenatal rhesus testing and blood cross matching
A patient has intermittent episodes of jaundice and anaemia. What does that indicate?
Haemolysis
A patient has intermittent episodes of jaundice and anaemia as well as splenomegaly. What test would you think would be diagnostic of your suspicions?
DAT Coombs test to diagnose Haemolytic Anaemia (specifically autoimmune anaemia)
A patient with jaundice and anaemia presents with their extremities (hands) with painful and blue areas. What antibody would you expect to find and what type of disease would this be?
IgM
=Chronic Cold Agglutinin Disease (CHAD)
WHICH IS A TYPE OF AUTOIMMUNE HAEMOLYTIC ANAEMIA
What age bracket usually would be seen to have Chronic Cold Agglutinin Disease (CHAD) ?
Elderly
What antibody would you expect to see in a patient with suspected warm autoimmune haemolytic anaemia?
IgG
What is the difference between warm and cold autoimmune haemolytic anaemia?
The temperature refers to when the antibodies are active.
-Cold is due to IgM antibodies present and activated in the cold
-Warm is due to IgG antibodies being present and activated at body temperature
Who is most commonly affected by warm autoimmune haemolytic anaemia?
Middle aged females
What are the causes of warm autoimmune haemolytic anaemia?
Remember mneumonic
Idiotic Pensive Neds Ruminate with Meth Limes
1.Idiopathic (usually)
2.Penicillin
3.Nsaids
3.Rheumatic conditions (e.g Lupus)
5.Methyldopa (used for pre-eclampsia i.e hypertension)
5.Lymphoid malignancy
What are the causes of cold autoimmune haemolytic anaemia?
Remember mneumonic
Ill Patients May Even Live
1.Idiopathic
2.Paroxysmal nocturnal haemoglobinuria
3. Mycoplasma
4. Epstein Baar Virus
5.Lymphoid malignancy
What is the management for warm autoimmune haemolytic anaemia?
-Treat underlying cause AND
1.Corticosteroids BUT TAKES 3WEEKS TO WORK
2.Splenectomy considered if not responding to corticosteroids
What are the causes of non-immune acquired haemolytic anaemia?
-Malaria, Clostridium Perfringens
-Acquired membrane defect : Paroxysmal Nocturnal Haemoglobinuria
-Mechanical Heart Valves
-Microangiopathic Haemolytic anaemia (fibrin deposition in capillaries)
-ITP (Idiopathic Thrombocytopenia Purpura)
-Burns causing injury
What is Paroxysmal Nocturnal Haemoglobinuria?
=Rare Acquired Haemolytic anaemia type that causes:
-Intravascular haemolysis
-Thrombosis (venous and arterial)
-Deficient haematopoiesis
What is the management for Paroxysmal Nocturnal Haemoglobinuria?
Supportive care
-intermittent blood transfusions
-long term anticoagulation
-Eculizumab (stops the antibody responsible)
-
In terms of presentation, what are the differences between a clotting and coagulation disorder?
Site:
-Clotting = bleeding within skin (bruising and purpura) or mucosal membranes (GI, uterine)
-Coagulation=bleeds involves joints (Haemarthroses) or Muscles (Haematoma)
Onset:
-Clotting= occurs spontaneously
-Coagulation = occurs within hours to days
What is Haemarthroses and how does it present?
Bleeding into articular spaces (between bones at the joints). Common in patients after an injury or with Haemophilia.
=swelling and pain at the joint region
What clinical disorder is a platelet count especially screening for?
Thrombocytopenia
What clinical disorder is a bleeding time especially screening for?
-Platelet dysfunction
-Von Willibrand Disease
What is a Activated Partial Thromboplastin Time looking especially for?
-For monitoring Heparin therapy, Warafin therapy, Haemophilia, Disseminated intravascular coagulation, Liver disease
What is the inheritance for Hereditary Haemorrhagic Telangiectasia?
Autosomal Dominant