ميحرلا نحمرلا للها مسبmedicinemosul.uomosul.edu.iq/files/pages/page_991378… ·  · 2016-03-12•CVS: No palpitation, no chest pain , dyspnea on exertion and

بسم اهلل الرمحن الرحيم

HISTORY • Name: Shamsah Mohammed Abdul-rahman

• Age: 75 years

• Occupation: Housewife.

• Residence: Mosul/Hmmam Alil.

• DOA: 30-10-2013.

• DOE: 6 -11-2013

CHIEF COMPLAINT: REDNESS AND SWELLING

OF RIGHT LEG FOR 7 DAYS DURATION.

• Hx of present illness: the condition started before 2 year when

she complained from severe headache all over her head not

associated with vomiting nor visual disturbance she was

admitted to hospital and MRI was done showing no

abnormalities and CBC also done that show increased PCV and

Hb level and diagnosed as Primary polycythemia she received

hydroxyurea with phlebotomies about 1pint/ month for 1 year

until her CBC was controlled then she took hydroxyurea

infrequently , before 1 year in Ramadan she fast and developed

fever, cough whitish sputum then she treated as out-patient with

antibiotics became well.

• In the last Ramadan she fast and stopped her

treatment and became unwell after that she

developed gradual redness and swelling with

pain and tenderness in her right leg and treated

for few days without improvement then she

consulted her physician who admitted her to

hospital.

REVIEW OF SYSTEMS:

• GIT: no loss of appetite, no vomiting, no hematemesis, no epigastric pain, but with constipation.

• CVS: No palpitation, no chest pain , dyspnea on exertion and no orthopnea.

• Respiratory system: S.O.B. on exertion ,mild cough with little whitish sputum, no hemoptysis, no chest pain.

• G.U.S: no hematuria, good urine output ,no dysuria or frequency, no loin pain.

• CNS: Headache , no visual disturbance, no insomnia, no fit, normal gait

• M. S.S: Weakness ,No Pruritus No Diaphoresis.

PAST HISTORY:

• PMHx No hypertension, no DM, no other chronic disease apart from PRV

• PSHx: Negative

• Social hx: Married , neither smoker nor alcoholic .

• Family hx: Negative of haematological diseases, low economic status.

• Drug Hx: infrequent Hydroxyurea tab, no drug allergy

GENERAL EXAMINATION:

• Old aged female ,conscious, not dyspnic,

afebrile, plethoric face not cyanosis, not

jaundiced, normal hair distribution , no

lymphadenopathy, no petechea but ecchymosis

at the site of cannula.

• The calf of her right leg is swollen, redness

tender with intact distal pulse.

VITAL SIGNS:

• Temp: 37.2oC

• RR: 16/min

• PR: 94 b/m

• BP: 140/100 mmHg

• sPO2: 97%

• Chest: normal VB, clear .

• Heart: NDR.

• Abdominal examination: Inspect: ; no distend abdomen, move normal with respiration, invert umbilicus. No dilated vein.

• palpation: soft abdomen, no palpable spleen the liver is not enlarged.

• Auscultation: normal bowel sound.

HAEMOSTATIC TESTS:

• Bleeding time (Ivy’s):

4.5 min (N.V. 2 -7 min)

• Prothrombin time: T: 16.0 sec

C: 13.0 sec

• INR :1.5

N.V. TEST

3.6 – 6.1mmol/L FBS: 5.1 mmol/L

136-155 µmol/L S. Sodium: 135 µmol/L

3.5 – 5.3 µmol/L S.Potassium 4.7 µmol/L

< 17 µmol/L Total serum bilirubin: 13 µmol/L

♀ up to 31/L S. A.L.T.: 10 u/L

♀ up to 32/L S. A.S.T.: 14 u/L

♀ 35 – 104 u/L S. Alkaline phosphatase 74 u/L

3.3 -7.5 mmol/L B. Urea: 8.3 mmol/L

♀ (53 – 97 µmol/L ) S. Creatinine: 91 µmol/L

♀ 155-357 µmol/L S. Uric acid: 435 µmol/L

ULTRA-SOUND OF THE ABDOMEN:

• Normal liver size and echogenicity ,normal biliary passages and portal veins

• Normal GB no stones.

• Both kidneys are normal size and echogenicity ,no stones .

• Normal pancreases

• The splenic size (length 13.5 cm X width 6.5cm) mild splenomegaly.

• Normal urinary bladder

• No masses or enlarged L.N.

DOPPLER ULTRASOUND OF THE RIGHT LEG

• Dilated right popliteal vein with positive thrombosis.

• Color void.

• Signal void.

• Conclusion: Doppler ultrasound images confirm the presence of deep venous thrombosis in the right popliteal vein. (D.V.T. of the vein of the right leg with thrombophlebitis).

THE POLYCYTHAEMIAS

• True polycythaemia refers to an absolute increase in

total body red cell volume (or mass more than of

expected for body mass or gender), which usually

manifests itself as a raised haemoglobin concentration

(Hb) and/or packed cell volume (PCV).

• Apparent (or relative) polycythaemia A raised Hb (or

PCV) due to a reduction in plasma volume, without an

increase in total red cell volume.

• True polycythaemia is further subdivided into

• primary polycythaemia (namely PV), a clonal

haematological disorders in PRV and germline mutation

in EpoR in inherted polycythemia.

• secondary polycythaemia, which results from an

increased erythropoietin drive, either in the presence or

absence of hypoxia.

• The red cell indices in iron deficient polycythaemia may

be indistinguishable from those of thalassaemia trait

(▼Hb &▲RBCC)but the RDW is more likely to be

elevated and there may be associated features that are

useful in the differential diagnosis such as neutrophilia,

basophilia, thrombocytosis and the presence of giant

platelets.

The diagnosis of polycythaemia is most commonly

suspected in a patient with an abnormally high

result on one or more of the following blood tests :

Hematocrit —. patients with a persistently raised

venous haematocrit (Hct)

(>0.52 males, >0.48 females for >2 months)

• Hemoglobin concentration —Polycythemia is

suspected when the Hb is >16.5g/dl or >18.5g/dl

in women and men, respectively.

THROMBOTIC COMPLICATIONS

Thrombosis is the most common serious complication (specially in

untreated PRV) which can be arterial, venous or microvascular.

This is due to:

1. Increased PCV leads to an increased blood viscosity,

2. Rheological abnormalities

3. Abnormal platelet – endothelial contact

4. Additionally, procoagulant changes in platelets (e.g. decreased

response to prostaglandin D 2), thrombocytosis and pre -

existing vascular disease

NEUROLOGICAL FEATURES

• The consequences of occlusive vascular lesions, the

sluggish cerebral blood flow secondary to the increased

PCV is thought to underlie features such as

headaches, drowsiness, insomnia, amnesia, tinnitus,

vertigo, chorea and even depression. Transient visual

disturbances also occur.

SKIN MANIFESTATIONS AND PRURITUS

• Plethora, dilated conjunctival vessels and rosacea - like

facial skin changes, Brown discoloration of the skin,

erythromelalgia and, rarely, Sweet syndrome

• Pruritus occurs in 25% of patients (sometimes severe)

specially after warm bath(aquagenic), it is attributed to

increased numbers of mast cells in the skin and to

elevated histamine levels, Pruritus is often relieved by

controlling the PCV.

SPLENOMEGALY

• splenomegaly is seen in 30 – 50% of cases of PV.

• HYPERTENSION AND GOUT

• Hypertension is common in patients with PV .

• hyperuricaemia, with gout seen in about 5% of cases.

LEUKAEMIC TRANSFORMATION

• The risk of developing acute leukaemia in PV patients treated

only with venesection is very small (1 – 3%).

• MYELOFIBROSIS:

• Progression to myelofibrosis occurs in around 10 – 20% of PV

cases at 15 years after diagnosis.