Friday, February 13, 2015

Long Case with Dato' Sapari

Patient 1

Mr. B, a 51-year-old Malay man with underlying Beta-Thalassemia intermedia and hypertension, presented with four days history of generalised body rash and one day history of gum bleeding. Platelet count on admission was 1,000.

Physical examination revealed a medium build man with no Thalassemic facies. Multiple petechiae noted at upper chest with ecchymosis at right cubital fossa. There was no gum hypertrophy or hepatosplenomegaly noted.

1. Provisional diagnosis?
Idiopathic Thrombocytopenic Purpura

2. How do you diagnose ITP?
It is a process of exclusion. First, determine that there are no blood abnormalities other than thrombocytopenia, no physical signs other than bleeding. Next, secondary causes should be excluded such as leukaemia, medications (heparin, quinine), SLE, APLS, cirrhosis, HIV, hepatitis C, congenital causes and others.

3. Investigations?
FBC, FBP, PT/APTT, LFT, RP, trace old notes and check for Hb electrophoresis result, bone marrow aspiration to look for increased production of megakaryocytes

4. Management?
Admit patient, insert branula, take blood investigations, refer haematologist, give high dose steroids (oral or IV), vital signs monitoring

5. How to differentiate bleeding in platelet and coagulation disorders?
Taken from this website

6. Indications of platelet transfusion in ITP? Taken from this website
Platelet transfusions are generally ineffective in patients with ITP – the transfused platelets are rapidly removed from the patient’s circulation. They are never useful when given for prophylaxis. 

In patients with serious haemorrhage who are being simultaneously treated with definitive therapy (IV methylprednisolone or other steroid, and IVIgG), however, platelet transfusions may be life saving. In these instances the doses required are enormous. While data are very few , an initial dose of 4 units is advised, repeated if necessary.

7. Peripheral blood smear in thrombocytopenia?
Taken from this website

Patient 2

Mr. F, a 16-year-old Malay teenager with underlying Ventral Septal Defect, presented with five days history of worsening dyspnea and three days history of haemoptysis. 

He defaulted follow up at National Heart Institute (IJN) after age of 5 due to financial problems. He was well until last year when he experienced dyspnea and chest pain on exertion. He was admitted to IJN last year and defaulted again after discharge. His symptoms re-occured since one month ago and worsened for the last five days before hospital admission.

Physical examination revealed a thin build teenager, small for his age, comfortably lying on hospital bed. He had central and peripheral cyanosis. Clubbing was noted at fingers and toes. BP 100/70, HR 80, T 37, RR 20.

Cardiovascular examination showed no raised JVP. Apex beat was heaving and displaced. Left parasternal heave was felt. Palpable thrill felt at tricuspid area with pansystolic murmur heard. It was moderate in intensity, grade 4/5, not accentuated by inspiration or expiration. Loud P2 was heard at pulmonary area.

1. Provisional diagnosis?
Pulmonary hypertension due to large VSD

2. Differential diagnoses?

  • Complex cyanotic congenital heart disease (CCCHD) eg. 
  • Eisenmenger + endocarditis (causing clubbing)
  • CCCHD + pulmonary hypertension + pneumonia (causing haemoptysis)

3. What is Eisenmenger's syndrome?
It's a condition whereby a large left-to-right shunt (VSD/ASD/PDA) causing severe pulmonary vascular disease and pulmonary hypertension > leading to reversal of the direction of shunting > cyanosis

Taken from UM X'Press

4. Complications of Eisenmenger's?

  • Hyperviscosity symptoms (headache, dizziness, visual disturbance) - due to polycythemia
  • Hemoptysis - due to pulmonary infarction or rupture of pulmonary arterioles or arteries
  • Cerebral infarction - due to paradoxical embolism and venous thrombosis
  • Cerebral abscess
  • Syncope - due to reduced cardiac output and atrial fibrillation
  • Right heart failure
  • Sudden death (cardiac arrhythmia)

5. Investigations?

  • FBC - polycythemia
  • ECG - right ventricular hypertrophy, atrial fibrillation
  • CXR - prominent central pulmonary arteries, reduced markings ("pruning") of peripheral vessels
  • ECHO - shunt, increased right ventricular pressure
  • Cardiac catheterization with pulmonary vasodilator (oxygen or nitric oxide) - assess reversibility of pulmonary hypertension (reversibility suggests benefit from surgical repair of defect)

6. Management?

  • Patient and family education on current condition, complications, recurrence in future if not treated, advice on drug usage, seek treatment of infection or hemoptysis occur, need to refer to IJN for cardiac surgery
  • Reduce pulmonary pressure by giving calcium channel blocker (Diltiazem) or PDE5 inhibitor (Viagra, Cialis, Levitra) or epoprostenol (pulmonary vasodilator)
  • Venesection to relieve hyperviscosity symptoms  

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