M5 MEQs /10/2016 Case 3: Ms Hui Bo Gao

Transcription

1 M5 MEQs /10/2016 Case 3: Ms Hui Bo Gao

2 Welcome to the amazing world of hematology J

3 Question 1.1: Ms Hui Bo Gao Ms Hui is a 30 years old female. She has no past medical history. She was referred from her GP with the presentation of 4 days history of fever and a rash. She has no other bleeding manifestations. On examination, her vitals was T38.5 BP 100/62, HR 80 RR22 SpO2 99% on RA. She looks comfortable. Examination was revealing of a rash over the limbs and trunk which is non-blanchable.

4

5 1.1 Question: Ms Hui Bo Gao Q1. Which are the 2 most important test will you order first? Full blood count Liver Panel Renal Panel HIV Test Dengue Serology PT/APTT Blood Cultures

6 A classic image of purpura

7 1.1 Question: Ms Hui Bo Gao Q1. Which are the 2 most important test will you order first? Full blood count looking for thrombocytopenia Liver Panel Renal Panel HIV Test Dengue Serology dengue rash usually appears when the fever lyses; this pt is still febrile with a rash PT/APTT looking for coagulopathy Blood Cultures The rest are not wrong but you are being asked to prioritise!

8 Small lesions (<0.5cm) Macule flat, cirumscribed, colored, non palpable Papule raised, solid and palpable Vesicle raised, palpable, clear fluid-filled Pustule raised, palpable, pus filled (leukocytes or keratin) Large lesions (>0.5cm) Patch large macule (flat non-palpable colored area) Plaque superficially raised, circumscribed solid area Nodule distinct large papule Bulla - large vesicle (blisters if epidermal layer completely sloughed off) Wheal firm and edematous plaque (edema of the dermis) Other Plaque/scaley papule Eschar Erosion/ulcer Purpura/petechia Plaque/smooth papule

9 Purpura is used to describe red purple lesions that result from the extravasation of blood into the skin or mucous membranes Non-blanchable Purpura may be palpable or non-palpable (flat/macular) Macular purpura is divided on 2 morphologies characterized by size and typically non-inflammatory Petechiae < 3mm Ecchymosis - > 5mm Palpable purpura: usually a sign of vascular inflammation

10 Question 1.2: Ms Hui Bo Gao ED bloods shows the following: Hb 6.9 TW 50.2 Plt 30 N.myelocytes Present Promyelocytes 90% Renal panel: U 7 Na 131 K 3.5 Bicarb 20 Cr 60 Liver Panel TBil 4 ALP 120 AST 55 ALT 42 PT 42 APTT 82 You review the patient in the ward, vitals T 38.9 BP 94/70 HR 80. She is still comfortable and does not have any bleeding manifestations.

11 Question 1.2: Ms Hui Bo Gao ED bloods shows the following: Hb 6.9 TW 50.2 Plt 30 N.myelocytes Present Promyelocytes 90% Renal panel: U 7 Na 131 K 3.5 Bicarb 20 Cr 60 Liver Panel TBil 4 ALP 120 AST 55 ALT 42 PT 42 APTT 82 You review the patient in the ward, vitals T 38.9 BP 94/70 HR 80. She is still comfortable and does not have any bleeding manifestations. TW 20, may be infection. TW 100, think leukaemia

12 Question 1.2: Ms Hui Bo Gao Q2. As the on-call HO, which of the following therapies would you initiate? (Choose 3 of 10) * Transfuse 1 pint PCT Transfuse platelets Referral to the haematologyon call Transfer to Medical ICA for monitoring Arrange for aphresis Start IV fluids 2L NS over 24H Start hydroxyurea1g TDS Repeat the FBC Do blood cultures and start empirical antibiotics Start dexamethasone

13 Don t know what is going on. Always go back to basics Transfuse 1 pint PCT Transfuse platelets Referral to the haematologyon call Transfer to Medical ICA for monitoring Arrange for aphresis --- you wouldn t start this yourself. Call haem! Start IV fluids 2L NS over 24H Start hydroxyurea1g TDS --- you wouldn t start this yourself. Call haem! Repeat the FBC Do blood cultures and start empirical antibiotics Start dexamethasone --- big no-no in acute leukaemia cases! Can cause tumor lysis.

14 Now contextualized Transfuse 1 pint PCT Transfuse platelets Referral to the haematologyon call Transfer to Medical ICA for monitoring --- patient is still well Arrange for aphresis Start IV fluids 2L NS over 24H Start hydroxyurea1g TDS Repeat the FBC --- you don t believe the lab is it? Do blood cultures and start empirical antibiotics Start dexamethasone

15 Some basic management principles Transfuse 1 pint PCT Transfuse platelets Referral to the haematologyon call Transfer to Medical ICA for monitoring Arrange for aphresis Start IV fluids 2L NS over 24H --- hydration is rarely wrong Start hydroxyurea1g TDS Repeat the FBC Do blood cultures and start empirical antibiotics --- probably quite safe Start dexamethasone

16 Question 1.3: Ms Hui Bo Gao You also noticed that the APTT/PT is prolonged. Your MO tells you to order other investigations to work up the cause. Q3. What investigation will you order next for this patient (Choose 2) 50% correction studies Direct coombs test D-Dimer Liver function test Fibrinogen Platelet function test Bleeding time

17 Question 1.3: Ms Hui Bo Gao You also noticed that the APTT/PT is prolonged. Your MO tells you to order other investigations to work up the cause. Q3. What investigation will you order next for this patient (Choose 2) 50% correction studies Direct coombs test D-Dimer Liver function test Fibrinogen Platelet function test Bleeding time This question is asking you, what do you think is the cause of prolonged aptt/pt? The investigation you choose is to confirm your suspicion. In this case coagulopathy is due to DIVC (happens in acute leukaemia or v sick pt) not due to liver disease or factor inhibitors or autoimmune dx.

18

19 Investigations in DIC: Prolonged of the prothrombintime (PT) Reflects reduced activity of the components of the extrinsic and common pathways Include factors VII, X, V, and prothrombin, which are the most frequently decreased clotting proteins in DIC Prolonged Activated partial thromboplastin time (aptt) Measures the intrinsic and common pathways of coagulation Is sensitive to deficiencies of factors XII, XI, IX and VIII Less sensitive than the PT to deficiencies of components of the common pathway

20 Investigations in DIC: Thrombocytopenia: Consumptive Plasma fibrinogen: Usually low in acute decompensated DIC Cavet: May be elevated as an acute phase reactant D-Dimer: high - indicative of fibrinolysis Peripheral Blood Film Thrombocytopenia Large platelets Fragmented red cells (schistocytes).

21 Question 1.4: Ms Hui Bo Gao The haematology reviews the blood film to confirm the diagnosis.

22 Question 1.4: Ms Hui Bo Gao Q4. What is the most likely diagnosis? * Acute myeloid leukemia Burkitts Lymphoma Acute promyelocyticleukemia Acute lymphoblastic leukemia Chronic myeloid leukemia

23 Question 1.4: Ms Hui Bo Gao Q4. What is the most likely diagnosis? * Acute myeloid leukemia Burkitts Lymphoma Acute promyelocyticleukemia Acute lymphoblastic leukemia Chronic myeloid leukemia

24 The answer was already in the stem! Promyelocytes: Very granular Weird looking big nuclei Auer rods

25 Question 1.5: Ms Hui Bo Gao The diagnosis of acute promyelocytic leukemia was made. The haematology registrars proceeds to order the appropriate treatment. Q5. What treatment was ordered? (Choose 1) * All trans retinoic acid Dexamethasone 4g TDS Hyper-CVAD IA3+7 Allogenic transplant

26 Question 1.5: Ms Hui Bo Gao The diagnosis of acute promyelocytic leukemia was made. The haematology registrars proceeds to order the appropriate treatment. Q5. What treatment was ordered? (Choose 1) * All trans retinoic acid Dexamethasone 4g TDS Hyper-CVAD IA3+7 Allogenic transplant

27 Management of APML Medical emergency: due to the risk of a life threatening bleed Treatment is started on clinical suspicion ATRA (All trans-retinotic acid) aka vitamin A Supportive management for bleeding complications

28

29 Question 1.6: Ms Hui Bo Gao The next day you were called to see Ms Tan for hemoptysis. The nurses informed you that she had coughed out blood 6 times in the last 1 hour, each time filling up a specimen blood (50ml). On review of the patient, Ms Tan was talking in full sentences. She does not complain of any breathlessness or chest pain. On examination, RR 24 SpO2 97% on RA. Heart sounds are heard with no addition murmurs or heart sounds. Lungs have reduced air entry at the bases, no additional sounds were heard.

30 Question 1.6: Ms Hui Bo Gao Q6. What will you do next in the management of this patient? Put patient on intranasal oxygen Consider elective intubation Patient is clinically stable, ask the nurse to inform you if further episodes occur Arrange for respiratory review and bronchoscopy Urgent thorax imaging with a CT thorax

31 Question 1.6: Ms Hui Bo Gao Q6. What will you do next in the management of this patient? Put patient on intranasal oxygen Consider elective intubation Patient is clinically stable, ask the nurse to inform you if further episodes occur Arrange for respiratory review and bronchoscopy Urgent thorax imaging with a CT thorax

32 Massive hemoptysis No clear definition: 500ml over 24H or 150ml over 1h Think of the anatomical dead space - the volume that is life threatening by virtue of airway obstruction (anatomical dead space is only ml) Medical emergency and should be addressed immediately. Initial priorities are stabilisation of the patient and protection of the non-bleeding lung

33 ABC first maintain airway patency: Asphyxiation is the most frequent complication of massive hemoptysis Assessment: History, Physical Examination, investigations Investigation: FBC/ PT/APTT/ CXR Initial Management: Oxygenation Lateral position ( if site of bleed is known - affected lung in dependent position) IV access Correct contributing coagulopathy/ thrombocytopenia Localize the source of the bleeding: Bronchoscpoy

34 Question 1.7: Ms Hui Bo Gao Patient was electively intubated and transferred to ICU. A bronchoscopy was done which showed diffuse bleeding. She was supported with blood products including platelets, fresh frozen plasma and cryoprecipitate. The bleeding finally stops and the ICU team considers extubation.

35 Question 1.7: Ms Hui Bo Gao Q7. Which of the following regarding blood transfusions is INCORRECT The typical effective dose of FFP is 10 to 15 ml/kg FFP is effective at treating mild coagulopathies characterized by INR of less than 1.85 Cryoprecipitate contains factor VIII, von Willebrand factor, factor XIII, fibronectin, and fibrinogen The risk for bacterial infection and sepsis is increased in patients receiving platelet transfusions compared with other blood products FFP is an acellular product

36 Question 1.7: Ms Hui Bo Gao Q7. Which of the following regarding blood transfusions is INCORRECT The typical effective dose of FFP is 10 to 15 ml/kg FFP is effective at treating mild coagulopathies characterized by INR of less than 1.85 Cryoprecipitate contains factor VIII, von Willebrand factor, factor XIII, fibronectin, and fibrinogen The risk for bacterial infection and sepsis is increased in patients receiving platelet transfusions compared with other blood products FFP is an acellular product

37 What can you transfuse Cellular products: RBC, platelets Plasma products: FFP, Cyroprecipitate, plasma derived products Approach Know your indication for transfusion Weigh the risk and benefits Remember: Blood products is a medication and is a public resource (donated)

38 Red blood cells Indication: improve the oxygen-carrying capacity of blood. Humans display many compensatory responses to maintain oxygen delivery, and no single hemoglobin trigger exists General guidelines: Hb >7 (Adjust base on patient s physiological state) Targets: Typically 250 to 300 ml with an expected increase in the hemoglobin of 1 g/dl (10 g/l) in a nonbleedingadult.

39 Platelets Indication: prophylactic and therapeutic for hemorrhage in patients with thrombocytopenia Options available: pooled or single donor 1 unit from a single donor = approx 4-5 pooled donors Targets: usually unit transfusion should bring up platelet count by at least k Note: Stored at room temperature the warmer storage conditions allow for proliferation of any contaminating bacteria

40 Fresh Frozen Plasma Indication: Prophylactic or therapeutic in patients with major coagulopathy, warfarin reversal, massive transfusion Contains all the blood clotting factors Targets: FFP volume ml; effective dosing 10-15ml/kg Note: Correction with FFP is not a linear phenomenoni Unlike PCT 1 pack PCT increases Hb ~1 g The amount of FFP required to bring INR from 10 to 9 is much less than that required to bring INR from 3 to 2.

41

42 Conclusions Haematology is fun Haematology is not scary!! Haematology is easy!!!