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Haem - pathology

Haematology - pathology

What tests are performed on donated blood to screen for HIV contamination? Anti-HIV-1, HIV-2 antibodies, and HIV-1 p24 antigen and nucleic acid testing to detect early infection
What is used as an anticoagulant in blood components? Citrate
How does citrate anticoagulent in blood components work? It binds calcium ions
What are used for preservatives in blood conponents? [3] Phosphate, dextrose, adenine
What are the available blood components? Red blood cells (RBCs), fresh frozen plasma (FFP), platelets (PLT), cryoprecipitate (cryo from FFP)
How many units of whole blood does the average adult have in his body? 8-10 units
On average, giving 1 unit of packed RBCs should raise the haemoglobin level by how much? 1 g/dL
What type of RBCs should be given to patients who have a problem with febrile reactions, or who will be chronically transfused? Leukocyte-reduced RBCs (since they decrease the risk of HLA auto-immunisation and the risk of transfusion reactions
What are the indications for giving patients FFP? [3] To replace coagulation factors, to reverse warfarin, for patients who have multiple coagulation factor deficiencies
What is a risk of FFP transfusion? Allergic reactions
What should be used as a volume expander in transfusions? [3] Albumin, crystalloid, or colloid (not FFP)
What is the usual dose of platelets? 1 unit per 10 kg body weight
What are apheresis platelets? Platelets collected from a single donor during an apheresis proceedure
What does cryoprecipitate contain? [4] Factor VIII, factor XIII, fibrinogen, and von Willebrand's factor
When might you administer cryoprecipitate? [4] Factor XIII deficiency, haemophilia A and von Willebrand's disease, fibrinogen deficiency, to prepare fibrin glue
What dose of cryoprecipitate should raise the level of fibrinogen by about 50 ml? 1 unit cryoprecipitate per 10 kg body weight
What iv fluid may be given with blood products? 0.9% normal saline or normal serum albumin (5%)
What will happen if you give dextrose 5% in water (D5W) with blood products? The blood will haemolyse because the fluid is hypotonic
What would happen if you give Ringer's lactate with blood products? Blood clots could form in the line because Ringer's lactate contains calcium the will bind with the anticoagulent (citrate)
In ABO blood groups, what is Landsteiner's rule? If you lack the RBC antigen, you make the antibody to it (type A antigen blood has anti-B antibodies)
What does Rh+ signify? The presence of the D antigen
What is Rh immune globulin? IgG anti-D antibodies in a purified preparation
What does the direct antiglobulin test detect? The presence of antibodies coating the RBC surface in vivo
What does the indirect antiglobulin test detect? RBC antibodies in the patient's serum (in vitro binding of IgG)
What is the most common metabolic side effect of massive blood transfusion? Alkalosis due to the citrate anticoagulant converting to bicarbonate in the liver
What correlates to the severity of a febrile non-haemolytic transfusion reaction? The number of leukocytes present in the blood component
What is the risk of FFP transfusions? Allergic/anaphylactic reactions
What causes allergic reactions in FFP transfusions? The patient has IgE antibodies against plasma proteins in the transfused blood
What type of patients will have anaphylactic reactions to FFP transfusion? Patients who are IgA deficient and have formed IgA antibodies
What can be done to prevent FFP anaphylactic reactions in IgA deficient patients? Use washed RBCs and platelets, or use plasma from other IgA-deficient patients
What causes TRALI (transfusion-related acute lung injury)? The donor's antileukocyte antibodies react with the recipient's leukocytes
What characterises TRALI clinically? [4] Pulmonary oedema, fever, tachypnoea, cyanosis
What type of infection are blood-transfusion recipients at greatest risk from? Bacterial contamination (not HIV or hepatitis)
What is the 'haemoglobin' on a CBC? The concentration of haemoglobin in the blood in g/dL
What is the hematocrit? A percentage of the total volume of erythrocytes relative to the total blood in a sample
What are the 3 major categories of anaemia? 1) normochromic, normocytic. 2) hypochromic, microcytic. 3) normochromic, macrocytic
Give examples of normochromic, normocytic anaemia Anaemia of chronic disease, haemolytic anaemias, acute haemorrhage, aplastic anaemias
Give examples of hypochromic, microcytic anaemias [2] Iron-deficiency anaemia, thalassaemia
Give examples of normochromic, macrocytic anaemia Vitamin B12 deficiency, folate deficiency
To evaluate anaemia of unknown origin, what must you remember to order? Reticulocyte count
What is the most common cause of iron depletion? Chronic blood loss
What are the microscopic findings of haemolytic anaemia? Spherocytes and schizocytes
What are spherocytes? RBCs that are misshapen in the sinusoids of the reticuloendothelial (RE) system
What are schistocytes? Poikilocytes arising from mechanical destruction of RBCs in the intravascular spaces
What cytoskeleton proteins are defective in hereditary spherocytosis? [2] Spectrin and ankyrin
What test is used to confirm hereditary spherocytosis? Osmotic fragility test
What is the treatment for hereditary spherocytosis? Splenectomy
What is the name for pancytopenia due to destruction or failure of myeloid stem cell, affecting all cell lines? Aplastic anaemia
What mutation occurs in sickle cell anaemia? Single amino acid replacement of glutamine with valine on the beta chains
What are some clinical findings associated with sickle cell anaemia? Anaemia, cholelithiasis, pain crisis, dactylitis, autosplenectomy
What causes a-thalassaemia? Underproduction of a-globin chain
What is Hb Bart's? All four a-globin genes are missing, resulting in hydrops fetalis
What causes b-thalassaemia minor? Underproduction of beta chain (heterozygote)
What causes b-thalassaemia major? Absence of beta chain
What 2 microscopic findings are associated with G6PD deficiency? Heinz bodies (membrane-bound precipitates that can lead to haemolysis) and bite cells (partially devoured cells)
How does disseminated intravascular coagulation (DIC) occur? Coagulation sequence is activated; microthrombi form; platelets, fibrin, and coag. factors are consumed, and fibrinolytic mechanisms begin
Name 2 common causes of DIC Obstetrical complications, sepsis
What lab findings characterise DIC? [4] High prothrombin time, PTT, and fibrin split products; low platelet count.
Which coagulation factor is deficient in haemophilia A? Factor VIII
Which coagulation factor is deficient in haemophilia B? Factor IX
What is the most common bleeding disorder? von Willebrand's disease
Which lab value is associated with the extrinsic pathway of the coagulation cascade? Prothrombin time (PT)
Which lab value is associated with the intrinsic pathway of the coagulation cascade? Partial thromboplastin time (PTT)
What is immune thromboplastic purpura (ITP)? An autoimmune disorder where autoantibodies form against platelets
What characterises ITP? Prolonged bleeding time with normal PT and PTT. Pinpoint haemorrhages, easy bruising, ecchymoses, low platelets, increased number of megakaryocytes in bone marrow
What population most often gets ITP? Young women aged between 20 - 40 years
What is thrombotic thrombocytopenic purpura? Widespread formation of hyaline thrombi and consumption of platelets that leads to thrombocytopenia and microangiopathic haemolytic anaemia
What might you see microscopically in TTP? Schistocytes: fragmented red cells
What is the pentad of symptoms seen in TTP? Fever, thrombocytopenia, microangiopathic haemolytic anaemia, neurological changes, renal failure
What causes Bernard-Soulier disease? A defect in platelet adhesion
What causes Glanzmann thrombasthenia? Problem with platelet aggregation
What type of leukaemia is most common in children? ALL (acute lymphoblastic leukaemia)
What type of leukaemia is most common in young adults (aged 15 - 40) AML (acute myeloblastic leukaemia)
What chronic leukaemia is most common in older people (aged 50 - 70)? CLL (chronic lymphocytic leukaemia)
What is associated with the Philadelphia chromosome? CML (chronic myelogenous leukaemia)
What is a urinary finding associated with multiple myeloma? Benxe Jones proteins (free light chains)
What genetic alteration is associated with Burkitt's lymphoma? t(8; 14) translocation, involving the c-myc oncogene
What genetic alteration is associated with follicular (B-cell) lymphoma? t(14; 18) and expression of bcl-2 involved in apoptosis
What is the most common type of Hodgkin's lymphoma? Nodular sclerosing Hodgkin's disease
Who usually gets Hodgkin's lymphoma? Young women
What is the characteristic cell of Hodgkin's disease? Reed-sternberg cell
What virus is associated with 50% of cases of Hodgkin's disease? Epstein-Barr virus
What lymphoma usually occurs in older adults? Diffuse large cell lymphoma
What aggressive lymphoma usually presents in children? Lymphoblastic lymphoma (T cells)
A gentleman has severe nausea and dizziness within an hour of eating shrimp. What type of hypersensitivity reaction is this? Type I hypersensitivity
A 4-year-old child has allergic rhinitis, eczema (atopic dermatitis), and asthma. What type of hypersensitivity is this? Type I hypersensitivity
A woman is Rh- and pregnant with her second Rh+ child. If she does not recieve anti-Rh immunoglobulin, what will happen? Erythroblastosis fetalis, or haemolytic disease of the newborn, will result since the mother will have anti-Rh antibodies in her blood that will cause haemolysis of the fetus' RBCs
A newborn baby shows signs of anaemia and jaundice within the first 24 hours of life. What might he have? Haemolytic disease of the newborn or possibly ABO blood group incompatability
A woman starts to feel exhausted and light-headed a week after beginning a course of penicillin. What is this? Autoimmune haemolytic anaemia, which is a type II hypersensitivity reaction to a drug (the body forms antibodies to the drug, and the antibody binds the drug on the RBCs, resulting in haemolysis)
A man develops an itchy, raised, red rash 2-3 days after using a new brand of laundry detergent. What is this? Contact dermatitis, which is a type IV hypersensitivity reaction
A man begins receiving a blood transfusion. He develops fever, chills, hypotension, and DIC. What happened? Acute haemolytic transfusion reaction. The patient received RBCs with an antigen that he had previously formed alloantibodies to
A child receives a bone marrow transplant from his healthy brother. He then develops a rash, jaundice, and diarrhoea. What is this reaction? Graft-versus-host disease (GVHD)
How can you prevent GVHD? Irradiating the donor cells to eliminate leukocytes
An alcoholic man presents with anaemia. Would you expect his MCV to be high or low? What is he probably deficient in? Expect him to have megaloblastic anaemia with a high MCV. He is probably deficient in B12 and/or folate
A 53-year-old man has been feeling tired recently. His Hb is 8.0. What should you be sure to order? Check for occult blood in the stool and order a colonoscopy
An elderly woman has lung cancer. What might characterise her anaemia of chronic disease? Decreased RBC lifespan, impaired iron metabolism, and possible refractoriness or lack of response to erythropoietin
A 7-year-old girl has an elevated white cell count, easy bruising, and fatigue. What should she be evaluated for? Acute lymphoblastic leukaemia
A 17-year-old girl notices a swollen node in her neck, and biopsy shows Reed-Sternberg cells. What disease is likely? Hodgkin's disease
What HLA allele is associated with ankylosing spondylitis? B27
What HLA allele is associated with postgonococcal arthritis? B27
What HLA allele is associated with acute anterior uveitis? B27
What HLA allele is associated with RA? DR4
What HLA allele is associated with chronic active hepatitis? DR3
Name the 4 types of transplant rejections Hyperacute (m - h), acute humoral (within first few months), acute cellular (within first few months), chronic (months to years and episodic)
What is the mechanism for hyperacute rejection? Humoral - there is a preformed cytotoxic antibody to the donor antigen, usually at the level of the vascular endothelium
What histological changes characterise hyperacute transplant rejection? Fibrinoid necrosis and thrombosis
What is the mechanism for acute vascular rejection? Humoral - antibodies to the donor antigen develop over time
What characterises acute humoral/vascular rejection? [4] Arteritis, necrosis, thrombosis, and neutrophilic infiltration
What is the mechanism for actue cellular rejection? Cell-mediated - the recipient's CD4+ and CD8+ respond to the donor
What histological changes characterise acute cellular rejection? [2] Lymphocytes/mononuclear cell infiltration; interstitial and tubular inflammation
What causes chronic rejection? Any disturbance in the ability of the host and graft to tolerate one another
What characterises chronic rejection? Vascular changes, especially intimal fibrosis
Describe GVHD The donor's lymphocytes in the graft react negatively to the recipient's cells
What characterises GVHD clinically? [3] Skin rash, jaundice, and diarrhoea
When is GVHD most often a problem? After bone marrow transplantation
If a person is deficient in polymorphoneutrophils (PMNs), what kind of infection is he most susceptible to? Staphylococcus, Aspergillus, Candida, and gram -ve bacteria
Deficiency in T cells makes you susceptible to what types of infections? [4] Mycobacteria, fungi, parasites, viruses
Deficiency in antibodies makes you susceptible to what types of infections? [2] Encapsulated organisms and viruses
Deficiency in complement makes you susceptible to what types of infections? Neisseria, especially Meningococcus
Created by: kuc