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Case PresentationCase Presentation

Professor Emeritus

Siddig A. Ismail UnitPresented by : Dr.Omer A/Mutaal

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Case HistoryCase History G.S.Idris is a lady of 70 years treated for

ischaemic heart disease for the last 2 years was admitted on 15.03.03 because of dyspnoea ,orthopnoea and ankles oedema progressing over the last 7 days .There was no haemoptysis, palpitation, chest pain or fever but arthalgia.

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Cont.Cont. Two months ago she developed rectal

bleeding for two days which was not noticed again after that time .

She is not known to be diabetic , hypertensive or asthmatic before.

No significant points in the family or social history.

She was on aspirin and ca channel blockers .

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Physical examinations revealed:Physical examinations revealed: ill, dyspnic, pale. Temp 37c No thyroid or lymph node enlargement. R.R 26/m J.V.P not raised P.R 100/m regular. P.P were intact. B.P 110/70. Gallop rhythm.No murmurs. Bilateral basal crackles. Spleen was 16cm below Lt. costal margin. Liver was enlarged of 8cm below Rt. costal margin. No ascitis. +ve L.L oedema. P.R.normal

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The problemThe problem

Jeopardized heart [due to I.H.D] faced with anaemia then went into congestive cardiac failure. What is behind the anaemia and huge spleen?

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Investigations showed: Investigations showed: C.B.C Hb%6.7g/dl R.B.C.s 4.25*10*6/mic.l H.C.T. 24.3% M.C.V. 57.2fl M.C.H. 15.8pg M.C.H.C. 27.6g/dl Platelets 1600*10*6/ mic.l E.S.R. 40mm/hr

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Cont.Cont. W.B.C. 41.7*103/ mic.l Neutrophils 84% Lymphocytes 3% Monocytes 1% Eosinophlis 9% Basophils 3% Retic. 1.7% Film reports very marked thrombocytosis

and leukocytosis.R.B.C.s showed marked hypochromia consistent with myeloproliferative disorder

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Cont.Cont. L.F.Ts. were normal. U&Es Urea 144mg/dl S.Na 135 mml/l S.Ka 4.3 m ml/l S.Ca 8.5mg/dl Serum uric acid 15.5mg /dl. R.B.G 125mg/dl U.G normal.

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Cont.Cont.

C.X.R. Cardiomegaly E.C.G :

Q waves in II – III – aVF (old in ferior MI ).

Vent . Ectopics .Echocardiography:

Good L.V.function EF 60% .

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Cont.Cont.

P.T test 15.6s cont.12-16s

I.N.R 1.6P.T.T. test 41s ( 31+/-6 ) Bleeding time 2:13

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U.S . abdomen: U.S . abdomen:

Enlarged liver with uniform coarse texture no focal lesion.

Contracted gall bladder with stones. Normal billiary system. Enlarged spleen with coarse texture. No lymphnode enlargement. Kidneys .Pancreas normal. No ascitis. No masses

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B.M.aspiration: B.M.aspiration: Marked hypercellular aspirate with abundant

large platelets clumps . Myelopoiesis is markedly hyperplastic with

predominance of mature forms . Megakaryocytes are abundant with nuclear

hyperlobulation. Erythropoiesis is depressed with megaloplastic

features .blast <5%.

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B.M. trephine: B.M. trephine:

Hypercellular marrow. Many megakaryocytes.Increase marrow fibrosis.

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Suggestion:Suggestion:

Essential Thrombocytosis.

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Management of essential thrombocytosis

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Minimizing risk factorsMinimizing risk factors

– Individualize the treatment based on risk factors for thrombohemorrhagic complications.

– Risk factors include the following: • Age 60 years or older • Prior history of thrombosis • Platelet count greater than 1.5 million per

microliter • Obesity • Smoking

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MedicationsMedications

o Low-risk patients (eg, lacking any of the above-mentioned risk factors): Observing these patients may be appropriate. Low-risk patients experience lower rates of thrombosis or bleeding. Generally, significantly increased thrombohemorrhagic risk is not associated with surgery or pregnancy in low-risk patients

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MedicationsMedicationso High-risk patients (eg, aged 60 y or older,

previous history of thrombosis): Provide cytoreductive therapy to best manage these patients. A randomized study comparing hydroxyurea versus observation in patients at high risk for thrombosis showed a marked decrease in thrombotic episodes in the hydroxyurea arm (Cortlazzo, 1995). Base the choice of the cytoreductive agent on patient factors (eg, age, child-bearing potential, cost, life expectancy, comorbidities).

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MedicationsMedicationso Intermediate-risk patients (eg, those who do not fit into

either high-risk or low-risk groups): Cytoreductive therapy or observation may help manage these patients. Recommend lifestyle modifications (eg, weight loss for obese patients, smoking cessation for smokers). The risk for hemorrhage and venous thrombosis increases in patients with platelet counts greater than 1.5 million per microliter. Administer aspirin to reduce the risk of major bleeding. These patients may be managed without drug intervention (as long as no concomitant aspirin use is present) or with cytoreductive therapy.

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MedicationsConsider administering hydroxyurea, anagrelide,

interferon alfa, or phosphorous-32 (common cytoreductive drugs that decrease the platelet count).

In addition, suggest low-dose aspirin, which may be useful in treating patients with symptoms of microvascular occlusion (eg, erythromelalgia).

Plateletpheresis may be useful in emergencies to achieve a rapid decrease in platelet counts in the setting of acute thrombosis and/or marked thrombocytosis

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