Untitled Deck Flashcards
1
Q
What is microcytic anaemia
A
Defined as small hypochromic RBC’s with low mean corpuscular volume ~ typically below 83 microns
2
Q
List some causes of haemolytic anaemia
A
Iron deficiency anaemia Insufficient intake Insufficient absorption Increased iron loss Increased iron requirements Sideroblastic anaemia Lead poisoning Congenital Chronic alcohol absue Anti TB medsMyelodysplasic syndromesAlpha and beta thalassaemia Anaemia of chronic disease ~ normocytic
3
Q
List some general signs and symptoms of anaemia
A
Tiredness Weakness Headches/dizzinessConjunctiva pallor TachycardiaIncreased RRDyspnoea Cold intolerance
4
Q
List some specific signs and symptoms of iron deficiency anaemia
A
Koilonychia Hair loss Pica ~ unusual dietary cravings Atrophic glossitis Angular stomatitis
5
Q
List some signs of lead poisoning (causes sideroblastic anaemia)
A
Motor Peripheral Neuropathy (e.g: Reduced grip strength, weakness in muscles/ absent reflexes)Bowel DisturbanceConfusion/personality changeMetallic taste in the mouthHaemolysis (clinically presenting with dark urine and pallor)Blue line on gum and dense metaphysial lines on radiograph
6
Q
Outline the investigations for iron deficiency anaemia
A
FBC Iron studies ~ serum iron(low), ferritin(low), transferritin saturation (low), high total iron binding capacityBlood film Peripheral blood smear ~ demonstrates hypochromic microcytic anaemia Reticulocyte count ~ low MCV ~ less than 83 microns
7
Q
What are the investigations for new iron deficiency anaemia
A
Colonscopy Oesoohageal gastroduodenectomy Need to be investigated for cancer
8
Q
Outline the invesitgations for sideroblastic anaemia
A
FBC Hb and MCV ~ low Iron studies ~ high ferritin, iron and transferrin Blood films Bone marrow examination
9
Q
Outline the management of iron deficiency anaemia
A
Treat underlying cause Replace iron ~ oral ferrous sulphate. IV iron, blood transfusion, iron rich diet Use erythropoietin if ferritin and transferrin saturation is normal
10
Q
Outline the management of sideroblastic anaemia
A
Treat underlying cause Pyridoxine
11
Q
What is macrocytic anaemia
A
When the RBC are bigger than normla but there is still low Hb It can be megaloblastic or normoblastic
12
Q
What is megaloblastic macrocytic anaemia
A
There is slow or impaired DNA synthesis which lead to delayed maturation of RBCs This leads to a large abrnomal RBC being produced There is also hypersegmented neutrophils on blood film
13
Q
List some causes of macrocytic anaemia
A
Megaloblastic: B12 deficiency ~ pernicious anaemia, dietary insufficiency and malabsorption Folate deficiency Normoblastic Alcohol - accompanied by raised yGGTReticulocytosis Hypothyroidism Liver diseaseDrugs Myelodysplasia
14
Q
List some causes of B12 deficiency
A
Vegan diet Post gastrectomy Illeal resection Crohns disease
15
Q
List some causes of pernicious anaemia
A
40 years oldBackground of auto-immune thyroid disease, vitiligo, T1DM and Addison’s diseaseFemale
16
Q
List some causes of a folate deficiency
A
Pregnancy (Increased demand)Poor diet + AlcoholCoeliac disease (Malabsorption)Drugs
17
Q
List some signs and symptoms of macrocytic anaemia
A
Pale skin & Conjunctival pallorTachycardia / palpitationsRaised respiratory rate & SOBTirednessHeadaches / DizzinessBeefy tongueWorsening of other conditions such as angina, heart failure or peripheral vascular disease
18
Q
List some signs and symptoms of B12 deficiency anaemia
A
Peripheral Neuropathy: Pins & needles (Paraesthesia), numbnessBeefy red sore tongueVisual ChangesWeakness, ataxia, spasticityMemory loss, confusion, irritability (mood)GlossitisAngular stomatitisLoss of vibration sense or proprioception
19
Q
Outline the investigations for macrocytic anaemia
A
FBC Haematinics ~ serum B12 and folate, MCV highBlood film TFT’s ~ hypothyroidism LFT’s Antibodies to intrinsic factor Markers of haemolysis ~ bilirubin etc
20
Q
Outline the management of megaloblastic anaemia
A
IM hydroxocobalamin (VitB12)Folic acid Dietary advice to increase B12 and folic acid You need to address the B12 deficiency first before folate replacement to avoid exacebating neuro symptoms and causing subacute degeneration of the spinal cord
21
Q
Outline the management of normoblastic anaemia
A
Treat the underlying cause like addressing alcohol consumption or support during pregnancy
22
Q
What are some complications of megaloblastic anaemia
A
Pernicious anaemia ~ increased risk of gastric cancer Folate deficiency ~ neural tube defects - all women should take 400mcg of folic acid until 12th week of pregnancy and those at high risk should start taking it from before conception
23
Q
What is sickle cell anaemia
A
Genetic condition where normal haemoglobin has a tendency to form abnormal Hb molecules upon deoxygenation leading to distorted RBC’s Autosomal recessive conditionHbSS - severe HbSC - milderHbAS - trait
24
Q
What causes sickle cell disease
A
Autosomal recessive inheritence ~ HbSS instead of HbAAHbSS ~ severe form HbSC ~ milder
25
Outline the pathophysiology of sickle cell disease
At the 6th position of the beta chain, glutamic acid is replaced by valine - decreases water solubility of deoxyHbIn its deoxygenated state, the HbS undergoes polymerisation to form cystals that cause polymers to form - RBC become sickle shaped The abnormal shape of RBC causes clotting in the microvasculature which precipitates a vaso-occulsive crisis leading to ischaemia and pain
26
What are the signs and symptoms of sickle cell disease
Symptoms dont show till after 4-6months of birth as there is transition from foetal Hb to adult Hb Painful vaso-occlusive crises - treat with pain killers Splenomegaly - due to extravascular haemolysis in the spleen Progressive anaemia Acute chest crises ~ tachypnoea, wheeze, couhg, hypoxia and pulmonary infiltrates on CXR
27
Outline the investigation for sickle cell disease
Newborn screening programme ~ heel prick on day 5 after birth Haemoglobin electrophoresis ~ definitive diagnosis Genetic testing Blood film ~ sickle cells, target cells, reticulocytosis with polychromsia, features of hyposplenism(Howell Jolly bodies, nucleated RBC)
28
Outline the management of chronic sickel cell disease
Chronic SCD Hydroxycarbamide/hydroxyurea ~ increases foetal Hb conc Vaccinations ~ pneumococcal polysaccharide every 5 yearsAntibiotic prophylaxis due to hyposplenisms Bone marrow transplant Blood transfusion, folic acid and iron chelation
29
Outline the emergency presentation of acute sickle cell crisis
Painful crisisVaso-oclusive crisis- happens in the coldSplenic sequestration crisis ~ RBC block splenic blood flow to cause splenomegaly and abdo pain, inc reticulocyte countAcute chest syndrome ~ vaso-occlusion in the pulmonary micorvasculature to cause infarcation of lung parenchyma Aplastic crisis ~ temporary loss of RBC production which is triggered by parvovirus B19 infection, reduced reticulocyte count and sudden fall in HbHaemolytic crisis ~ fall in Hb is related to rasied reticulocytes and prehepatic hyperbilirubinaemia
30
Outline the emergency management of acute sickle cell crisis
IV analgesia High flow oxygen IV fluids Antibiotics Blood transfusion Exchange transfusion ~ remove blood and replace with donor blood
31
List some complications of sickel cell disease
Sickle cell crisis Acute chest crisis Anaemia Avascular necrosis in large joints Stroke Pulmonary HTCKD Hyposplenism ~ sickle cells get stuck in the spleen and undergo phagocytosis to cause splenic congestion and splenomegaly - compromised splenic function therefore prone to infections
32
What is thalassaemia
Group of recessive inherited disorders characterised by abnormal Hb production
33
What are the 2 types of thalassaemia
Alpha ~ defect in the 4 genes for alpha globin chain Beta globin chain defect results in beta thalassaemia major or minor
34
Outline the pathophysiology of alpha thalassaemia
Caused by nonfunctioning copies of the 4 alpha globin genes on chromosome 16If they have 2 defective genes ~ pt has alpha thalassaemia trait If they have 3 defective copies ~ pt has symptomatic haemoglobin H disease (microcytic anaemia, haemolysis, splenomegaly)If they have all 4 defective copies - they have hydrops fetalis which is incompatible with life
35
What are the signs and symptoms of alpha thalassaemia
Juandice Fatigue Facial bone deformities Splenomegaly
36
What are the investigations for alpha thalassaemia
FBC ~ shows microcytic anaemia with disproportionately low MCVHb electrophoresis
37
Outline the pathophysiology of beta thalassaemia
If the pt has one abnormal of the 2 beta globin copies ~ beta thalassaemia minor If they have one abnormal gene and one deleted gene or 2 abnormal genes ~ beta thalassaemia intermediaIf they have 2 deleted beta globin copies ~ beta thalassaemia major - complete absence of beta globin synthesis
38
List the signs and symptoms of beta thalassaemia major
Severe symptomatic microcytic anaemia at 3-9 months Frontal bossing Maxillary overgrowth HepatosplenomegalyExtramedullary hematopoiesis Failure to thrive
39
Outline the investigations for beta thalassaemia major
FBC ~ microcytic anaemia - disproportiante decrease in Hb and MCV - MCV much more decreased than ironHb electrophoresis DNA testing Blood film ~ hypochromic microcytic cells, target cells, nucleated RBCs Pregnant women are offered screening tests
40
Outline the management of thalassaemias
Blood transfusions ~ beta major should have regular transfusion (2-4wks) of packed RBCs for the rest of their lives Splenectomy Stem cell transplant/ bone marrow transplant Reduce the risk of iron overload in beta major pts ~ give iron chelating agents like desferrioxamine
41
List some complications of beta thalassaemia
Iron overload toxicity ~ due to recurrent blood transfusions Cardiac arrythmias Acute sepsis Liver cirrhosisEndocrine dysfunction
42
What happens to the bone marrow due to thalassaemia
RBCs are more fragile and break down more easilyThe spleen filters the defected RBCs and destroys them → splenomegalyBone marrow expands to compensate for the anaemia → increased susceptibility to fractures
43
What is haemolytic anaemia
The premature destruction of RBCs resulting in low Hb conc
44
What are the 2 types of autommine HA
Warm Cold
45
What is warm AIHA
Haemolysis occurs at warm temperatures via antibody reaction IgG mediated Spleen tags these cells for splenic phagocytes ~ this measn its an extravascular haemolytic disease
46
What causes warm AIHA
Idiopathic SLELymphoproliferative neoplasms Drugs
47
What is cold AIHA
At lower temps, the AB's attach to the RBC;s to clump them together ~ agglutinationThe complement system is activated and the RBCs are destroyed IgM mediated ~ causes direct intravascular haemolysis
48
What causes cold AIHA
Neoplasms Post infections - mycoplasma pneumoniaeIdiopathic
49
List the key features of autoimmune haemolytic anaemia
Anaemia Increased reticulocyte Low haptoglobin High LDH and uncongugated bilirubin Blood film ~ spherocytes and reticulocytes Positive Coombs tests ~ direct antiglobulin test
50
Outline the management of autommine haemolytic anaemia
Steroids ~ prednisalone Immunosuppresive drugsRituximab for cold AIHASplenectomy Blood transfusion
51
What is ALL
A type of haemotological malignancy characterised by uncontrolled proliferation of immature lymphoid precursor cells within the bone marrow It is the most common cancer in childhood
52
What causes ALL
Caused by the abnormal proliferation of lymphoid progenitor cells like B cells It is associated with Down syndrome Assoicated with the Philadelphia chromosome ~ exchange between chromosome 9 and 22
53
List the subtypes of ALL
Precursor B cell ALL T cell ALLB cell ALL
54
List some signs and symptoms of ALL
Fatigue Abnormal bleeding/bruising Infections Bone pain Painless lymphadenopathy Hepatosplenomegaly Cranial nerve palsies Testicular enlargement
55
Outline the investigations for ALL
Bone marrow biopsy ~ diagnostic FBC - leukocytosis (WBC)Blood film + bone marrow analysis ~ presence of 20%+ lymphoblasts/blast cells is diagnostic Periodic acid Schiff stain Lymph node biopsy
56
What symptoms would warrant an urgent FBC to assess for leukaemia
Pallor Persistent fatigue Unexplained fever Unexplained persistent or recurrent infection Generalised lymphadenopathy Unexplained bruising Unexplained bleedingUnexplained petechiae Unexplained hepatomegaly
57
Outline the chemotherapeutic regimen for ALL
Induction therapy ~ vincristine, prednisalone, L-asparaginase+daunorucibin Consolidation therapy ~ high/medium dose drugs in blocks over several weeks Maintenance therapy ~ daily mercaptopurine combines with weekly methotrexate for 2-3 years CNS prophylaxis ~ intrathecal methtrexate
58
What is the supportive care for ALL
Blood/platelet transfusion IV fluids Allopurinol ~ prevent tumour lysis syndrome Insert subcut port system or Hickman line for IV access
59
List some complications of ALL
Infections Bleeding CNS involvement Chemotherapy related toxicities
60
What is AML
A haemotological malignancy characterised by uncontrolled proliferation of myeloid precursors in the bone marrow leading to bone marrow failure and the accumulation of immature WBC's in the peripheral blood Its the common acute leukaemia in adults
61
What causes AML
Deletion in chromosome 5 or 7 Can result of a transformation from a myeloproliferative disorder
62
List some signs and symptoms of bone marrow failure
Anaemia Thrombocytopenia Neutropenia Splenomegaly Bone pain
63
Outline the investigations for AML
Bone marrow biopsy ~ diagnostic and will show a high proportion of blast cells, lots of immature myeloid cells Blood film ~ high proportion of blast cells but also Auer rods in cytoplasm Neutropenia and thrombocytopenia and anaemiaFBC Cytochemistry CytogeneticsImmunophenotyping if its difficuly to distinguish between AML and ALL
64
Outline the medical management of AML
Chemotherapy of daunorubicin and cytarabine but chemo starts with induction period and then consolidation therapy Bone marrow transplant
65
Outline the supportive care for AML
Blood/platelet transfusion IV fluids Allopurinol ~ prevents TLSInsert subcut port line or Hickman line for IV accessProphylactic antimicrobials
66
What is CML
Its a myeloproliferative neoplasm characterised by the presence of the Philadelphia chromosome which leads to the formation of the BCR-ABL1 gene This altered gene results in an abnormal tyrosine kinase enzyme which causes the uncontrolled proliferation of myeloid cells in the bone marrow Its the most common in middle aged patients
67
List some signs and symptoms of CML
Massive splenomegaly Tiredness Bleeding Gout May report a sense of fullnessSystemic symptoms ~ fever, sweating and weight lossHyperleukocytosis symptoms ~ visual disturbances, confusion, palpitation and deafness
68
What are the 3 phases of CML
Chronic phase ~ lasts around 5 years, asymptomatic and is diagnosed incidentally due to raised WCCAccelerated phase ~ abnormal blast cells make up the vast majority of cell in the bone marrow and blood, this is where they become symptomatic Blast phase ~ even higher proportions of blast cells, with severe symptoms, pancytopenia and often fatal
69
Outline the investigations for CML
FBC ~ shows leukocytosis, neutrophilia, eosinophilia thrombocytosis/penia and anaemia Peripheral blood smear ~ mature myeloid cells, and multiple basophils and oesinophils Blood film ~ increased granulocytes at different stages of maturation and thrombocytosis Bone marrow analysis Genetic testing for the BCR-ABL1 gene or Philadelphia chromosome (9,22)
70
Outline the management of CML
Imatinib ~ first line drug and its a tyrosine kinase inhibitor Hydroxycarbamide ~ used to help normalise the blood count by stopping cells from making and repairing DNAInterferon alpha Allogenic bone marrow transplant
71
What is CLL
A haematological malignancy characterised by accumulation of mature monoclonal B lymphocytes in the blood, bone marrow and lymphoid tissues These abnormal B cells are often slow growing and crowd out healthy cells Most common leukaemia in adults It may cause warm autoimmune haemolytic anaemia
72
List some signs and symptoms of CLL
Typically asymptomatic Non tender lymphadenopathy Hepatosplenomegaly BleedingInfection B symptoms ~ weight loss, night sweats and fever
73
Outline the investigations for CLL
Blood film ~ smudge cells, smear cells Immunophenotyping bone marrow biopsy ~ CD5 and CD23+ve FBC ~ lymphocytosis - high lymphocyte count Bone marrow biopsy
74
What is Richters transformation
The transformation of CLL into high grade B cell lymphoma
75
Outline the management of CLL
Asymptomatic ~ close observation Chemotherapy and steroids ~ rituximab + fuldarabine + cyclophophamide Stem cell transplant Support care ~ transfusion, IV human immunoglobulin if they have recurrent infection
76
What are the complications of CLL
AnaemiaHypogammaglobulinaemia ~ causes recurrent infections Warm autoimmune haemolytic anaemia Transformation to a high grade non Hodgkin B cell lymphoma
77
List some complications of chemotherapy
Failure to treat cancerStunted growth and development in children Infections Neurotoxicity Infertility Secondary malignancy Cardiotoxicity Tumour lysis syndrome
78
What is tumour lysis syndrome
When tumour cells are destroyed, all of thier contents are released into the bloodstream
79
What are the characteristics of tumour lysis syndrome
High uric acid ~ can form crystals anywhereHigh potassium ~ cardiac arrythmiasHigh phosphateLow calcium
80
How do you manage tumour lysis syndrome
Rigorous hydration Correct electrolyte imbalances - may need dialysisManage hyperuricaemia with allopurinol or rasburicaseUse raburicase if they are in active TLSAllopurinol is prophylaxis for TLSEnsure good hydration and urine output before chemotherapy
81
List the signs and symptoms of tumour lysis syndrome
Dysuria and oliguria Abdo pain Weakness N&V Muscle cramps Seizures Cardiac arrythmias Joint swelling/gout
82
What is lymphoma
Type of cancer that affects lymphocytes inside the lymphatic system The cancerous cells proliferate insdie the lymph ndoes causing the lymph nodes to become abnormally large ~ lymphadenopathy
83
What is Hodgkins lymphoma
The malignant proliferation of lymphoctes characterised by Reed-Strenberg cells ~ large cells that is binucleated
84
List some risk factors for Hodgkins lymphoma
HIV EBV Immunosupresion Cigarette smoking
85
List the signs and symptoms of Hodgkins lymphoma
Non tender unilateral lymphadenopathy ~ commonly cervical/supraclavicular nodes B symptoms ~ weight loss, pruritus, night sweats Compression of surrounding structures to cause symptoms Alcohol induced painful lymphadenopathy
86
What is the staging of Hodgkin lymphoma
Ann Arbor staging syndrome Stage I – involvement of a single nodal groupStage II – involvement of two or more nodal groups on the same side of the diaphragmStage III – involvement of nodal groups on both sides of the diaphragm - above and belowStage IV – disseminated disease with involvement of extralymphatic organs (eg. the bones or lung)A/B refers to whether they have B symptoms or not like night sweats or fever
87
Outline the investigations for Hodgkins lymphoma
Lymph node biopsy ~ diagnostic and will show Reed sternberg cells FBC ~ eosinophilia, normocytic anemiaBloods ~ raised LDLPET CT
88
Outline the management of Hodgkins lymphoma
Chemotherapy DBVD ~ doxorubicin, bleomycin, vinblastin, dacarbazine BEACOPP ~ bleomycin, etoposide, doxorucibin, cyclophosphamide, vincristine, procarbazine, prednisalone RadiotherapyChemoradiotherapy Haemopoietic cell transplantation
89
What are the subtypes of Hodgkins lymphoma
Nuclear sclerosing ~ good prognosis Mixed cellularity ~ good prognosis Lymphocyte predominate ~ best prognosisLymphocyte depleted ~ worst prognosis
90
List some complications of Hodgkins lymphoma
Metastasis to breast and lungs
91
What is non Hodgkins lymphoma
Includes all the lymphomas without Reed-Sternberg cells More common than Hodgkins lymphoma
92
List some risk factors for non Hodgkins lymphoma
HIV EBV H.Pylori Hep B or C Exposure to pesticides Exposure to trichloroethylene FHxImmunodeficiency states
93
List some signs and symptoms of non Hodgkins lymphoma
Painless symmetrical lympadenopathy B symptoms ~ fever, night sweats, weight lossExtra nodal diseasesHepatomegaly + splenomegaly
94
Outline the investigations for Non hodgkins lymphoma
Excisional node biopsy ~ gold standard Bloods ~ LDL raised, thrombocytopenia,, pancytopenia, lymphocytosis Blood film ~ nucleated red cells and left shift (early WBC precursors) Bone marrow biopsy - atypical lymphoid cells and irregular nucleus and high mitotic rate CT CAP and PET to stage
95
Outline the management of non Hodgkin lymphoma
Depends on the specific subtype Watchful waiting Chemotherapy R-CHOP-21 ~ rituximab, cyclophosphamide, doxorucibin, vincristine and prednisalone for 21 days Radiotherapy All pts recieve flu/pneumococcal vaccine Neutropenic pts ~ abx prophylaxis
96
What are the 3 main types fo non Hodgkins lymphoma
Diffuse large B cell lymphoma ~ associated with Hep CBurkitts lymphoma ~ associated with EBV, malaria and HIV, painless lump in older 65'sMALT lymphoma ~ associated with H pylori
97
Outline gastric MALT lymphoma
Arises from B lymphocytes in marginal zone Associated with H pylori Has good prognosis Signs ~ abdo pain, N&V, anaemia, paraproteinaemia, extensive lymphocytes found on biopsy of mass Managed by eradicating H pylori
98
Outline Burkitts lymphoma
2 types ~ endemic (african) and sporadic Caused by c-myc gene translocation HIV is assocated with the sporadic form EBV (herpes virus 4) associated with endemic form Diagnosed by starry sky appearance, nucleated RBC, left shiftManagement ~ chemotherapy
99
List some complications of non Hodgkins lymphoma
Bone marrow infiltration SVC obstruction Metastasis Spinal cord compression Side effects of chemo
100
What is myeloma
Haematological malignancy where there is clonal proliferation of plasma cells - plasma cell dyscrasia Multiple myeloma ~ myeloma affects multiple areas of the body
101
Briefly outline the pathophysiology of multiple myeloma
Abnormal proliferation of plasma cells which secrete monoclonal antibodies or paraprotein and M proteins into the serum adn urine Deficiency of functional antibodies - causes relative hypogammaglobunaemia
102
List some risk factors for multiple myeloma
Old age Male Black african ethinictyFHx of haematological malignancy Obesity MGUS
103
List the signs and symptoms of multiple myeloma
CRAB HAIB HyperCalcaemia Renal impairment Anaemia Bone pathology ~ back pain Hyperviscosity Amyloidosis Infection Bleeding + bruising
104
Outline the investigations for multiple myeloma
FBC ~ normocytic anaemia, thrombocytopenia, leukopenia U&E's ~ raised urea, creatinine, calcium, normal/high phosphate and normal alkaline phosphate ESR Blood film ~ rouleaux formation Serum or urine protein electrophoresis ~ raised IgG/IgA paraprotein or raised Bence Jones protein - diagnostic Serum free light chain assay Bone marrow aspirate and biopsy ~ diagnostic Whole body MRI Skeletal survey Skull x-ray ~ raindrop skull
105
Outline the management of multiple myeloma
Conservative management ~ regular follow up unless signs of active diseaseInduction therapy ~ bortezomib, thalidomide, dexamethasone Followed by high dose melphalan as a conditioning regimen Followed by an autologoud stem cell transplant Erythropoietin for anaemiaAfter completion of treatment - pt monitered every 3 months with bloos tests and electrophoresis to check for relapse If relapse occurs ~ bortezomib monotherapy Myeloma bone disease ~ bisphosphonates and radiotherapy
106
What are the X-ray findings in a pt with myeloma
Well defined lytic lesions Diffuse osteopenia Abnormal fractures Pepper pot skull ~ multiple lytic lesion in the skull
107
What is leucocytosis
Increase in the number of WBCs
108
What are the 5 types of leucocytosis
Neutrophilia LymphocytosisMonocytosisEosinophilia Basophilia
109
List some causes of neutrophilia
Bacterial infection Inflammation Drug Pregnancy StressSmoking
110
List some causes of monocytosis
Chemo/radiotherapy Chronic infections Malignant diseaseMyelodysplasia
111
List some causes of lymphocytosis
Acute viral infectionChronic infectionsLeukaemiasLymphomas
112
List some causes of eosinophilia
Drug reactionsAllergies Parasitic infection Skin disease Malignant disease Adrenall insufficiency
113
List some causes of basophilia
Myeloproliferative disease Viral infection IgE mediated hypersensitivity reaction Inflammatory disorders
114
List some signs and symptoms of leucocytosis
Fatigue Fever Dyspnoea Splenomegaly
115
What are the investigations for leucocytosis
FBC ~ increased WCCBlood film Bone marrow biopsy
116
Outline the management of leucocytosis
Antibiotics if infection Antihistamine if allergic reaction Medication to reduce stress or anxiety Anti inflammatory medication Inhalers for asthma IV fludis Leukapheresis ~ procedure to quicklt reduce WCC Cancer treatment
117
What is neutropenia
Low neutrophil count <1.5 x109
118
What is agranulocytosis
Neutropenia with depleted basophils and eosinophils
119
List some causes of neutropenia
Severe sepsis Viral infections Drugs Infiltration of the bone marrowHaematological malignancies ~ myelodysplastic malignancies and aplastic anaemia Hypersplenism SLE Radio/chemotherapy B12, iron, folate deficiencies
120
List some signs and symptoms of neutropenia
Often are just symptoms of an infection Fever Fatigue Sore throat Swollen lymph nodes Pain, swelling or rash at affected site Diarrhoea
121
What are the investigations for neutropenia
FBCFind the cause ~ cultures, lumbar puncture, imaging etc
122
What are the types of neutropenia
Mild ~ 1-1.5 x 109Moderate ~ 0.5-1 x 109 Severe ~ <0.5 z 109
123
Whats the normal neutrophil count
2.0-7.5 x 109
124
Outline the management of neutropenia
Use granulocyte colony-stimulating factor ~ stimus the production of neutrophil in bone marrow Oral abx If febrile ~ quinolone with co amoxiclav Infection control ~ put the pt in a side room and use PPE
125
What is leukaemoid reaction
Increase in WBC >50,000 which is caused by an underlying disease that isnt leukaemia
126
List some causes of leukamoid reaction
Severe illnessBurns Drugs Haemorrhage Malignancy Intoxication ~ ethylene glycol
127
List some signs and symptoms of leukamoid reactions
FatigueFever Weakness
128
What are the investigations for leukamoid reaction
Excluce leukaemia FBC ~ WCC increase Blood film ~ increased neutrophil precursors, cytoplasmic toxic granulation, Dohle bodiesBone marrow aspiration/biopsy
129
How do you treat leukamoid reaction
Treat underlying cause
130
What is pancytopenia
Combination of anaemia, thrombocytopenia and leukopenia (RBC, platelets and WBC)
131
What can cause pancytopenia
Decreased marrow haematopoetic function Inherited bone marrow failureIncreased destruction of blood cells peripherallyImmune destruction of blood cells
132
List some signs and symptoms of pancytopaenia
RBC ~ lethargy, pale skin, other symptoms of anaemiaWBC ~ fevers, infection Platelets ~ easy bruising and bleeding, petechiae
133
Outline the investigations for pancytopaenia
FBC ~ decreased WBC, RBC and platelets Peripheral blood film Bone marrow biopsy
134
Outline the management of pancytopaenia
Red cell transfusion Platelet transfusion Platelets ~ granulocyte colont stimulating factors
135
What is neutropaenic sepsis
Defined as a neutrophil count <0.5x109 in a patient having anti cancer therapy wth one of the following:Temp greater than 38 or Symptoms and signs of sepsis Medical emergency!
136
What is the main cause of neutropaenic sepsis
Chemotherapy ~ mainly 7-14 days after
137
What pathogens mainly cause neutropaenic sepsis
Gram -ve ~ E.coli, P.aeruginosa, Klebsiella Gram +ve ~ Staph epidermidis, Staph aereus, Strep pneumoniaeFungi ~ Candida, aspergillus
138
List some signs and symptoms of neutropaenic sepsis
Often asymptomatic as they dont have enough WBC to mount a response but may have:Tachycardia Hypotension Fever ~ may not have this Sore throatImmunosupressed
139
List some medications that can cause neutropaenic sepsis
Chemotherapeutic drugs Clozapine Hydroxychloroquine MethotrexateSulfasalazine Quinine Infliximab Azathioprine + allopurinol
140
What are the investigations for neutropaenic sepsis
FBC ~ WCC decreased CRP/ESR raised Blood culturesCXR Serology and PCR Sputum, urine and stool samples Swabs
141
Outline the management of neutropaenic sepsis
IV broad spectrum abx ~ piperacillin with tazobactam Prophylactic fluroquinalones Sepsis 6 ~ IV fluids, oxygen, blood cultures, lactate measures, urine output If they are low risk ~ oral abx Daily measures of fever and baseline bloods until the patient is apyrexial and neutrophil count is >0.5x109
142
What is haemophilia
X linked recesive inheritied bleeding disorders
143
What causes haemopholia A
Deficiency in Factor VIII
144
What causes haemophilia B
Lack of factor IX
145
List some signs and symptoms of haemophilia
Spontaneous deep and severe bleeding into soft tissues, joints and muscles Haematomas Bruisings Intercranial haemorrhage Cord bleeding Other bleeding in unusual sites like gums, GIT etcFailure to walk in toddles due to bleeding into the joints
146
What are the investigations for haemophilia
Factor VIII or IX assay APTT ~ prolonged Prothrombin time is normal Genetic testing
147
Outline the management of haemophilia
Minor bleeding ~ desmopressin Major bleeds ~ replace clotting factors (VIII or IX) by IV infusions - recombinant factors
148
What is the emergency management of haemophilia
Infusion of the affected factor ~ VIII or IX Desmopressin to stimulate the release of von Willebrand factor and Factor VIII Antifibrinolytics
149
What is Von Willebrands disease
Inherted bleeding disorder characterised by a reduced quantity or function of von Willebrand factor Most commonly inherited bleeding disorder
150
What are the 3 types of Von Willebrands disease
Type 1 ~ partial reduction in vWF ~ most common Type 2 ~ abnormal form of vWF ~ abnormal binding to factor VIII or defective platelet adhesion Type 3 ~ total lack of VWF ~ most severe
151
What causes Von Willebrands disease
Most are due to an autosomal dominant inheritance Type 3 is autosomal recessive
152
Briefly outline the pathophysiology of Von Willebrand disease
This protein normally links platelets to the exposed endothelium and stabilises clotting factor VIII Dysfunction or deficiency leads to an increased risk of bleeding
153
List the signs and symptoms of Von Willebrands disease
Hx of unusually easy, prolonged or heavy bleeding Menorrhagia/postnatal haemorrhage Epistaxis Excess or prolonged bleeding from minor wounds or post operatively Easy bruisingEpistaxis GI bleeding
154
Outline the investigations for Won Willebrands disease
Clotting tests ~ normal PT and TT Prolonged APTT and bleeding time Platelet count is normal vWF level and activity assay Factor VIII activity is normal but decreased becasue its bound to VWF
155
Outline the management of Von Willebrands disease
Mild bleeding ~ desmopressin (first line)Mild bleeding + heavy mensturation ~ tranxamic acid or mefenemic acid, COCP or mirena coil Major bleeds + surgery ~ infusion of vWF-containing factor VIII concentrate
156
What are thrombophilias
Conditions that predispose patients to develop blood clots due to dysregulation of the coagulation system They can be inherited or acquired
157
What can cause inherited thrombophilias
A deficiency in a natural anticoagulant ~ antiphospholipid syndrome, antithrombin III deficiency or Protein C or S deficency Gain of function polymorphism ~ Factor V leiden (most common) or prothrombin gene mutation
158
What is factor V leiden
Mutation in factor V causes it become ressitant to inactivation by protein c
159
List some signs and symptoms of thrombophilias
Recurrent venous clots Calf swelling ~ DVT Pain or tenderness along deep vein system ~ DVT Chest pain ~ PEBreathlessness ~ PEHypotension ~ PETachycardia and tachypnoe ~ PE
160
What is anti thrombin III deficiency
Anti thrombin normally inihibits factor IIa, Xa, IXa and XIaDeficiency increases the risks of thrombosis Homozygousity causes death in utero Heterzygosity increases the risk fo VTE by 50 folds
161
What is protein C deficiency
Protein C inactivates clotting factors V and VIIIInactivating mutation in the protein C increass the risk of thrombosis
162
What is protein S deficiency
Protein S is a vitamin k dependent co factor for the anticoagulation activity of protein C Deficiency is associated with the high risk of VTE in young people
163
List some risk factors for VTE
Age Pregnancy Active malignancy Antiphopsholipid syndrome Previous PE or DVT Being bed bound over 5 days Dehydration Recent surgery COCP+HRT Smoking Long haul air travel Obesity
164
How does increasing age increase risk of VTE
As you increase in age, the levels of activated factor VII, IX and X increases and well as increased levels of factor XII, fibrinogen and D dimer which all increase risk of thrombosis
165
How does pregnancy/post partum increase risk of VTE
Pregnancy causes a fall in protein S and increase in fibrinogen, factor VIII and vWF which results in activated protein C resistance - clots can form
166
How does malignancy increase risk of VTE
The pt is in a prothrombic state and there is greater activation of the coagulation system due to TF expression and fibrinolytic activity Malignant cells will also release cytokines and will interact with endothelial cells and platelets
167
What is antiphospholipid syndrome
An autoimmune disorder characterised by arterial and venous thrombosis, adverse pregnancy outcomes and raised levels of antiphospholipid antibodies
168
List the main features of antiphospholipid syndrome
Clots ~ usually venous thromboembolisms rather than arterial Livedo reticularis ~ mottled, lace-like appearance of skin in lower legs Obstetric loss ~ recurrent miscarriages or premature birthsThrombocytopenia Cardiac valvular disease
169
Outline the investigations for antiphospholipid syndrome
One or more of the following positive blood tests are needed on 2 occasions, 12 weeks apart to diagnose it: Anti-cardiolipin AB'sAnti-beta2-GPI AB'sPositive lupus anticoagulant assay
170
Outline the management of anti phospholipid syndrome
Primary prophylaxis ~ low dose aspirin Secondary prophylaxis - if they had 1 VTE - life long warfarin with target INR between 2-3If recurrent VTE - lifelong warfarin and increase target INR to 3-4 Arterial thrombosis is treated with lifelong warfarin with target INR 2-3Remember, warfarin is teratogenic and in pregnancy use alternative anticoagulation like low molecular heparin
171
What are the VTE prophlaxis treatment available
If a pt is at increased risk of VTE they should recieve low molecular weight heparin such as enoxaparin Anti embolic compression socks can be used ~ avoid in peripheral arterial disease Venous VTE ~ avoid oestrogen containing contraception, HRT and long period of immobility Arterial ~ control CV risk factors
172
What is TTP
A condition where tiny thrombi develop throughout the small vessels and use up the platelet supplyIt is characterised by microangiopathic haemolytic anaemis and thrombocytopenic purpura
173
List some causes of TTP
Idiopathic Pregnancy HIV Drugs Post infection Tumours SLE
174
Outline the pathphysiology of TTP
Dysfunction in the ADAMTS13 proteinThis protein normally inactivates vWF and reduces platelet adhesion and clot formation Deficiency means overactivation of vWF - clots form - RBC breakdown and causes anaemia
175
List some signs and symptoms of TTP
Neurological abnoramlity - seizuresFever Renal failure Thrombocytopenia MAHA on blood filmPT and APTT are normal
176
Outline the investigations for TTP
Platelets ~ low Hb ~ low Bilirubin ~ high LDH/troponin ~ high Urea and creatinine ~ high Blood film ~ schistocytes
177
Outline the management of TTP
Plasma exchange ~ gold standard Steroids Rituximab
178
What is INR
A measure of how long it takes blood to form a clotIt has to be monitored in pts who are on anticoagulants
179
What is the target INR for patients on anticoagulants
2-3
180
What is a high INR
It means blood too thin which means there is a risk of excessive bleeding An INR >4.5 increases the risk of major haemorrhage
181
What is a low INR
It means blood is too thick which means the risk of clotting/thrombosis INR <2 increases the risk of thromboembolism
182
List some causes of high INR
Overdose of anticoagulant medication Drug interactions Herbal products Increase in alcohol consumption Low vitamin K Infection
183
List some signs and symptoms of high INR
Any evidence of bleeding Overt blood lossBruising
184
Outline the investigations for high INR
History Dosing history of anticoagulants Concurrent illness Change in diet/lifestyle/medications History of any falls/injuries History of blood loss Bloods FBC ~ check for signs of anaemia and infection Clotting screen ~ check of any other abnormalities Do a CT head if you are worried by intercranial haemorrhage
185
Outline the management of high INR greater than 8 if there is major bleeding
Stop anticoagulants Administer IV vitamin K 5mgAdminister prothrombin complex ~ use FFP is not available
186
Outline the management of high INR greater than 8 if there is minor bleeding
Stop anticoagulation Administer IV vitamin K 1-3mg Repeated INR after 24hrs and see if they need further vitamin KRestart warfarin when INR is below 5
187
Outline the management of high INR >8 with no bleeding
Stop anticoagulants Administer IV or oral vitamin K 1-3mg Repeat INR after 24 hours
188
Outline the management of high INR >5 and no bleeding
Withold 1-2 doses of anticoagulants Review maintenance dose of anticoagulant ~ may need to be lowered
189
When can you restart warfarin
When the INR is less than 5
190
What is DIC
It is the inappropriate activation of clotting cascade resulting in thrombus formation and depletion of clotting factors and plateletsIt is a microangiopathic haemolytic anaemia
191
List some causes of DIC
Sepsis Trauma/burns Malignancy Infections Vascular conditionsObstetrics complications
192
Outline the pathophysiology of DIC
TF is on many cells but not normally in contact with general circulation ~ it is exposed after tissue damage When activated, TF binds coagulation factors triggering after extrinsic pathways and instrinsic pathways Therefore after major trauma when there is tissue damage DIC can occur as TF is released
193
List the signs and symptoms of DIC
Excessive bleeding FeverHypotension Confusion Petechiae
194
Outline the investigations for DIC
Blood tests: FBC ~ shows thrombocytopeniaDecreased fibrinogen Increased prothrombin time and APTTRaised D dimer Blood film shows schistocytes (broken RBCs) due to microangiopathic haemolytic anaemia
195
Outline the management of DIC
Traet the underlying causeCyroprecipitate or FFP firstBlood products transfusion ~ platelets, FFP to replace coagulation factors and cyroprecipitate to replace fibrinogen In some cases anticoagulation therapy may be necessaryIf its due to septic shock - initiate sepsis 6
196
What is massive blood loss
Sudden and continuing blood loss of greater than 2 litres
197
What 4 products can be readily transfused
RBC'sFFP PlateletsCyropreciptate
198
What blood group is the universal donor for packed RBC's
O
199
What are the indications for packed red cell transfusions
Symptomatic/chronic anaemia Major haemorrhage Cases where infusing large volumes can lead to circulatory overloadHeart failure ~ give packed RBC and furosemide
200
What is the universal donor for FFP
AB
201
What is in FFP
Clotting factors Antibodies Albumin
202
What are the indications for FFP
Bleeding due to multi factor deficiencies Raised PT/APTTProphylactic correcting clotting factors before high risk surgery
203
What is the main contraindication for FFP
Dont use a volume expander
204
What is the indication for platelets
Thrombocytopenia
205
List the contraindications for platelet transfusions
Heparin induced thrombocytopenia Chronic bone marrow failure Thrombotic thrombocytopenic purpura Autoimmune thrombocytopenia
206
What is in cyroprecipitate
Contains factor VIII, vWF and fibrinogen
207
List the indications for cyroprecipitate
Clinically significant haemorrhage Uncontrolled bleeding Allows large amount of clotting factor to be administered in a small volume Low fibrinogen
208
What are the indications for prothrombin complex concentrate
Emergency reversal of anticoagulanttion in pts with severe bleeding Prophlactic treatmetn for pts having emergency surgery
209
What are cell saver devices
They collect the pts own blood lost during surgery and reinfuse ir There are two main types ~ either washes the blood cells before reinfusion or do not wash blood prior to re infusion
210
What is the main advantage of cell saver devices
Avoids the use of infusing donor blod therefore reduces the risk of blood bourne infection
211
What are the 2 types of transfusion reactions
AcuteChronic
212
What are the types of acute transfusion reactions
Acute haemolytic Febrile non haemolytic Allergic Transfusion related acute lung injury Transfusion associated criculatory overload
213
Outline an allergic reaction to blood transfusion
Cause ~ reaction to foreign components in transfusion Features ~ urticaria, pruritus and anaphylaxis Management ~ stop transfusion, saline adrenaline and oxygen if needed, antihistamine
214
Outline acute haemolytic transfusion reaction to a blood transfusion
Cause ~ incompatible blood given ang IgM mediated RBC destructionFeatures ~ fever, hypotension, abdo/chest pain, agitation Management ~ stop transfusion, saline resus and treat DIC, send bloods for direct Coombs test
215
Outline febrile non haemolytic transfusion reaction to a blood transfusion
Cause ~ antibodies to WBC HLAFeatures ~ fevers and chills Management ~ slow the transfusion and give paracetamol
216
Outline transfusion related acute lung injury
Cause ~ increased vascular permeability caused by host neutrophils which are activated by donor blood Features ~ pulmonary oedema, ARDS, hypoxia, hypotension, fever, white out on CXRManagement ~ stop the transfusion, give saline and treat ARDs
217
Outline transfusion associated circulatory overload
Cause ~ fluid overloadFeatures ~ pulmonary oedema and hypertensive Management ~ slow the transfusion and give furosemide
218
List some delayed reactions to blood tranfusions
Delayed haemolytic transfusion reaction Transfusion associated graft-verses-host disease Post transfusion purpura
219
Outline delayed haemolytic transfusion reaction
Cause ~ exaggerated response to foreign antigen Features ~ jaundice, anaemia, fever and usually post 5 days tranfsuion Management ~ fluids
220
Outline transfusion associated graft-verses-host disease
Cause ~ donot lymphocytes attacking recipient body Carries a high risk of mortality but is rareManagement ~ immunosuppresion with IV steroids
221
Outline post transfusion purpura
Cause ~ immune response against platelets Features ~ bleeding Management ~ IV immunoglobulins
222
How is iron overload treated
Subcutaneous desferrioxamine
223
Outline the Billingham criteria to diagnose graft vs host disease
Graft tissue contains immunologically functioning cells Recipient and donor are immunologically different Recipient is immunocompromised
224
What is polycythaemia
Its an increase in haematocrit, red cell count and haemoglobin concentration It can relative, primary (polycythaemia rubran vera) or secondary
225
What causes relative polycythaemia
Falsely elevated haemoglobin secondary to a low plasm volume such as dehydration, excess diuretic use, diarrhoea etc
226
What is absolute polycythaemia
Plasma volume is normal and red cell mass is raised
227
List some causes of secondary polycythaemia
Its due to inappropriate rise in erythropoetin COPD Altitude Obstructive sleep apnoea Excess erythropoeitin ~ cerebellar haemnagioma, hepatoma, uterien fibroids
228
What is primary polycythaemia
There is excessive and uncontrolled erythrocytosis that is independent to erythropoetin levels Most cases are associated with mutation in the JAK2 gene which cuases uncontrolled production of blood cells esp RBC
229
How do you differentiate between absolute and relative polycythaemia
Use red cell mass studies In men the total red cell mass is >35ml/kg and in women >32ml/kg
230
List some signs and symptoms of polycythaemia
Splenomegaly Portal hypertension Low platelets Thrombosis ~ arterial and venousRaised RBC Low WBC ErythromelalagiaFacial redness
231
What is polycythaemia rubra vera
A myeloproliferative disorder caused by clonal proliferation of marrow stem cell leading to increase in red cell volume as well as overproduction of neutrophils and platelets
232
What causes polycythaemia rubra vera
Mutation in JAK2 gene
233
List some risk factors for polycythaemia rubra vera
Budd-chiari syndrome Greater than 40 years old
234
List the features of polycythaemia rubra vera
Hyperviscosity Pruritus after hot bathsLeg swellingSplenomegaly Haemorrhage Plethoric appearance ~xs redness in eye conjucntiva May be accompanied with high neutrophil and platelets
235
Outline the investigations for polycythaemia
FBC and blood film ~ raised haematocrit, neutrophils, basophils and platelets JAK2 mutation test Serum ferritin Renal and liver function tests Vit B12 levels Bone marrow biopsy
236
Outline the management of polycythaemia
Venesection ~ first line Aspirin ~ to reduce risk of thrombotic events Cytoreductive therapy if venesection doesnt work ~ 1st line is hydroxyurea and in younger patients its interferon Allopurinol for gout
237
What is essential thrombocythaemia
Chronic myeloproliferative disorder caused by dysregulated megakaryocytes proliferation causing an abnormally high platelet count > 400x109
238
What causes essential thrombocythaemia
Due to JAK2 V617F mutation
239
Why does essential thrombocythaemia increase the risk of bleeding
All the excessive platelets will use up the free vWF which means not enough will be available at the site of injury
240
List some risk factors for essential thrombocythaemia
Female 50-70 years old
241
List some signs and symptoms of essential thrombocythaemia
Thrombosis Bleeding Splenomegaly Erythromelalgia ~ discoluration and pain in the extremities Hyposplenism Livedo reticularis Systemic features ~ fatigue, weight loss etc
242
Outline the investigations for essential thrombocythaemia
FBC ~ increases platelet count Bone marrow biopsy ~ unusually large megacaryocytes Low iron Genetic testing for JAK2 V617F mutation
243
Outline the management of essential thrombocythaemia
Hydroxyurea ~ reduce platelet count Interferon alpha for younger patients Low dose aspirin ~ reduce thrombotic risk
244
What is anaemia of chronic disease
It is generally normocytic, normochromic anaemia Characterised by low Hb levels, decreased RBC production and altered iron metabolism
245
List some causes of anaemia of chronic disease
Due to chronic inflammation of an underlying disease: Malignancy Chronic infections Connective tissue disease Autoimmune conditions Surgery
246
Outline the pathophysiology of ACD
Chronic inflammation produces inflammatory cytokines like IL-6Raised IL6 will stimulate the release of hepcidin from the liver which reduces iron absorption by reducing the activity of ferroportin - iorn channel This leads to decrease in Hb production There is iron stored in the body, but your body is unable to utilise it therefore cuases anaemia
247
Lists some signs and symptoms of ACD
Pallor Fatigue SOB on exertion Headache/dizziness
248
Outline the investigations for ACD
FBC ~ low Hb and low MCVBlood film ~ normocytic normochromic Serum iron studies ~ low serum iron, high ferritin and low transferritin saturation low TIBC CRP and ESR ~ raised
249
Outline the management of ACD
Treat underlying cause IV iron therapy RBC transfusion Erythropoietic agents
250
What is haemochromatosis
Autosomal recessive genetic condition resulting in iron overloadCaused by mutation in the HFE gene on both copies of chromosome 6
251
List the signs anf symptoms of haemochromatosis
Chronic tiredness Joint pain Swollen jointsBronze skin pigmentation Testicular atrophy Erectile dysfunction Amenorrhoea Cognitive symptomsHepatomegaly Hypogonadotrophic hypogonadism due to iron deposits in the pituitary therefore cannot release gonadotrophins
252
Outline the investigations for haemochromatosis
Bloods ~ deranged LFTs, raised serum ferritin and raised transferritin saturation, raised serum iron, decreased total iron binding capacity Genetic testing for HFE mutation ~ most common diagnostic investigationMRI imaging if the brain and heart ~ iron deposition Liver biopsy ~ Perl stain confirms increased iron stores
253
Outline the management of haemochromatosis
Venesection ~ remove some blood every week Desferrioxamine ~ iron chelating agent Avoid fruit juices Monitor serum ferritin and transferritin
254
List some complications of haemochromatosis
Secondary diabetes Liver cirrhosisEndocrine and sexual problems Cardiomyopathy Hepatocellular carcinoma Hypothyroidism Chrondrocalcinosis ~ calcium pyrophosphate deposits in the joint
255
What is HDN
An autommune condition where a rhesus negative mum becomes sensitised and develops antibodies against her rhesus positibe blood cells of her baby in utero
256
List some events in which a RhD-ve mum can develop anti RhD ab's
Antepartum haemorrhage Abdominal trauma Placental trauma Invasive uterine procedure Ectopic pregnancy Intrauterine death Transfusion of RhD+ve blood Delivery
257
Outline the pathophysiology of HDN
During a sensitisation event, the mothers blood mixes with the RhD+ve antigens from the foetus and develops IgG AB's agaisnt the RhDSo if the mother has a 2nd RhD+ve child, the anti Rhd IgG can cross the placenta and cause haemolysis of Rh+ve RBC's of the baby
258
List some signs and symptoms of HDN
Hydrops foetalis ~ foetal oedema in 2 diff compartmetns seen on USYellow coloured amniotic fluid due to excess bilirubin Neonatal jaundice and kernicterus Foetal anaemia Hepatomegaly or splenomegalyHeart failure
259
Outline the invesitgations for HDN
Test maternal blood type Maternal serum RhD ab screen DAT/Coombs test Kleihauer test
260
Outline the management of HDN for the mothers
Anti-D abs at 28 weeks and at birth of the RhD+ve baby Give extra dose of anti-D prophylaxis immediately to pts who had a sensitising event Early delivery if needed
261
Outline the management of HDN for the neonate
Exchange transfusion to manage high bilirubin UV phototherapy for high bilirubin
262
What is haemorrhagic disease of the newborn
Vit K deficiency bleeding It can early, classic or late
263
What casues haemorrhagic disease of the newborn
Deficiencies in Vit K as it cannot cross the placenta This means there is a deficiency of some clotting factors
264
What clotting factors require vit K for its production
Factor II, VII, IX and X
265
What are the risk factors for haemorrhagic disease of the newborn
Breast fed babiesMaternal use of anti epileptics
266
List some signs and symptoms of haemorrhagic disease of the newborn
Easy bruising Intercranial haemorrhage Internal bleeding Juandice
267
Outline the investigations for haemorrhagic disease of the newborn
FBC Clotting screen CXR or US for inter throacic bleed CT or MRI for intercranial bleed
268
Outline the management of haemorrhagic disease of the neonate
Vit K supplement ~ subcut offered routinely at birth FFP if severe bleeding
269
What is the main complication of haemorrhagic disease of the newborn
Haemorrhagic shock
270
What is immune thrombocytopenia
Autommune condition characterised by the reduction in the number of circulation platelets It is a type II hypersensitivity reaction where hte spleen produced IgG/IGgM ab's againt glycoprotein IIb/IIIa or Ib-V-IX complex (anti platelet antiboides)
271
List some signs and symptoms of immune thrombocytopenia
Bruising Petechial/purpuric rash Mucocutaneous bleedingBlood in urine or stool BleedingHistory of recent viral infection
272
Outline the investigation for immune thromboytopenia
FBC ~ isolated thrombocytopenia Blood filmBone marrrow examination ~ only if lymphadenopathy, splenomegaly, abnormal WBC or failrue to resolvePT and APTT is normal
273
Outline the management of immune thrombocytopenia
Usually self limiting ~ wait and watch If platelet count very low or significant bleeding:Oral steroids - first line IV steroids IV immunoglobulins - second linePlatelet transfusion ~ avoided unless life threatening bleedingSplenectomy
274
What is the definition of a major bleed
intracranial, retro-peritoneal, intraspinal, intra-ocular, pericardial or intramuscular bleeding with compartment syndrome
275
What is the reversal agent for heparin
Protamine sulphate
276
What is the reversal agent for Warfarin
Vit K and prothrombin complex concentrateFFP only to be used if PCC is unavailable
277
What is the emergency management of a major bleed
Stop the anti coag Correct haemodynamics Give the reversal agent depending on what anti coag they were on
278
What is sideroblastic anaemia
The body produced enough iron but is unable to put it into haemoglobin
279
List some causes of normocytic anaemia
anaemia of chronic diseasechronic kidney diseaseaplastic anaemiahaemolytic anaemiaacute blood loss
280
How do Reed Sternberg cells look
Large cells that are multinucleated or have a bilobed nucleus Prominent eosinophilic inclusion like nuclei - owl's eye appearance
281
What is the reversal agent for apixaban and rivaroxaban
IV andexanet alfa
282
What bloods would you do for myeloma
FBC ~ anaemia Peripheral blood film ~ rouleaux formation U&E's ~ high urea and creatinine suggesting renal failure Bone profile ~ hypercalacaemia
283
What will protein electrophoresis show
Raised monoclonal proteins in the serum Bence Jones proteins in the urine
284
What will bone marrow aspiration show
Significantly riased plasma cells
285
What is the long term management of sickle cell disease
Hydroxyurea to increase HbF levels Pneumococcal palysoccharide every 5 years
286
How does acute chest syndrome present
Dyspnoea Chest pain Cough Hypoxia New pulmonary infiltrated on CXR
287
What is the complication of polycythaema rubra vera
AML
288
What is the reversal agent for dabigatran
Idarucizumab
289
What conditions have target cells
Sickle cellThalassaemia Hyposplenism Liver disease
290
What conditions have tear drop poikilocytes
Myelofibrosis
291
What conditions have spherocytes
Hereditary spherocytosis Autoimmune haemolytic anaemia
292
What conditions have basophillic stippling
Lead poisoning Thalassaemia Sideroblastic anaemia Myelodysplasia
293
What conditions have Howell Jolly bodies
Hyposplenism
294
What conditions have Heinz bodies
G6PD deficiency Alpha thalassaemia
295
What conditions have schistocytes - helmet cells
Intravascular haemolysis Mechanical heart valve DIC
296
What conditions have pencil poikilocytes
Iron deficiency anaemia
297
What is this
Spherocytes
298
What is this
Tear drop Poikilocytes
299
What is this
Target cells
300
What is this
Heinz bodies
301
What is this
Howell Jolly bodies
302
What is this
Basophillic stippling
303
What is this
Pencil poikilocytes
304
What is this
Schistocytes - helmet cells
305
How can you differentiate between haemophilia and VWB disease
Haemophilia ~ bleeding into joints more common, also very very rare in women cos its X linked VWD ~ mennorhagia and GI bleeding more common
306
What symptoms indicate Richters transformation
Lymph node swelling Fever withoht infection Weight loss Night sweats Nausea Abdo pain
307
How would aplastic crisis present
Caused by infection with parvovirus Sudden fall in Hb Reduced reticulocyte count
308
How would sequestration crisis present
Increased reticulocyte count Splenomegaly Abdominal pain
309
List the signs and symptoms of haemolytic anaemia
Fatigue Pallor JuandiceSplenomegaly Dark urine Gallstones Leg ulcers SOBPalpitations
310
What will a Hb electrophoresis show in beta thalassaemia major
HbA2 and HbF are produced No HbA produced
311
What will Hb electrophoresis show in sickle cell anaemia
HbS produced
312
What is the bleeding pattern of haemophilia
Deep bleeding like - joint bleeding, muscular haematomas, big bruises etc
313
What is the bleeding pattern in VWB disease
Platelet pattern Cutaneous petechiae and purpura Epistaxis Mucosal bleeding - GI and mennorhagia
314
Who are pts with low risk ETP
Age less than 40 ANDPlatelet count <1500No history of thrombosis or haemorrhage No CVD risk factors
315
How are pts with low risk ETP treated
Aspirin alone
316
Which pts are high risk with ETP
Aged over 60 OR Platelet count >1500Previous history of thrombosis or haemorrhage Diabetes or hypertension
317
How are high risk ETP pts treated
Hydroxycarbamide/hydroxyurea AND aspirin
318
How are intermmediate risk ETP pts treated
Hydroxycarbamide and aspirin or just aspirin alone
319
How can you distinguish between IPT and TTP
ITP will present with isolated thrombocytopenia and and some signs of bleeding/bruising like petechiae and purpura TTP will present will more specific symptoms like fever, neuro symptoms etc
320
What is APML
Acute promyelotic leukaemiaRare type of AML caused by translocation between chromosome 15 and 17 resulting in disruption of retinoid acid receptorMarked increase in promyelocytesCan present with DIC
321
