Sickle Cell Crisis- VOC

16 JUL 2010

Sickle Cell Disease with Vaso Occlussive Crisis
    
Authors:
Dr.Mohamed Kabeer K.K[MEM- PGY 1]
Dr.Ramkumar S.[MEM- PGY 1]
Dr.Binu Kuriakose C [MEM- PGY 1]
Dr.Sajid Majeed [PGFEM- PGY 2]
Dr. Venugopalan P.P (HOD, EM)

A 21 year old male , a known case of sickle cell disease for the past 15 years with frequent episodes of pain crisis and on

exchange transfusions monthly (stopped 6 months ago)

Symptoms:
Presented with
Severe generalized pain ( Pain score – 10/10)
Priapism

Allergies: None

Medications: Hydroxyurea 500mg OD
               Warfarin 5mg OD
               Penicillin V 250mg BD
               Monthly exchange transfusion

Past medical/surgical history:
    Pulmonary embolism 5 years ago
    Acute chest syndrome 3 years ago
    DVT  1 ½ years ago
    Recurrent priapism and VOC’s
Social History : Non alcoholic ,no substance abuse

Airway: Patent
Breathing: Respiratory rate: 16/min
           Air entry bilaterally equal
Circulation: Pulse: 96/min regular
              BP: 80/50 mm of Hg
                  Normal Cappillary refill
Disability : Fully concoius Normal Pupil, No focal neurologic deficit
Exposure - No rashes
          
Vitals: Temp- 96.50F
    Pulse- 96/min regular
    BP- 80/50 mm of Hg
    Respiratory rate: 16/min
    SpO2: 100% with 4L of O2

Head to toe examination:
Thin built, short stature
    Pallor +
    Icterus +
    Chest : AEBE, Normal vesicular breath sounds
CVS: S1 S2 normal, systolic murmur over apex,No rubs
P/A: soft, non-distended
        Liver border felt 4cm below right midclavicular line
        No splenomegaly
        BS +
External genitalia: priapism +
Extremities :No edema, Pulses +
Neurology : Concious ,Oriented,Cran.Nerves Normal ,Motor ,sensory and Reflexes-Normal
No signs of Meningeal Irritation.

Differential diagnosis:
    Sickle cell anemia with VOC
    Hemolytic anemia
    

Labs:
    CBC – Hb: 7.2g%
        WBC count: 34,600/cmm
        DC: Polymorphs-40
                Lymphocytes-69
                Basophils-8
                Monocytes-1
    Reticulocyte count – 13.16%
    RBC – 2.14 x 106 /ml
    LDH - 795 U/L
    CPK – 296 U/L
    PT-35.3
    INR – 3.07
    aPTT – 56.1 sec
    URE -  Urobilinogen ++
        Bile pigment +
        RBC – 25/µL

Diagnosis: Sickle cell disease with Vaso Occlusive Crisis

Consultaion: Medicine,Surgery and Hematology

Treatment:
    Pain control-Morphine
    Oxygenation
    Hydration
    Hydroxyurea
    Blood transfusion/Exchange transfusion
    Aspiration and irrigation of corpora cavernosa
ED disposal - Admited in Medical ward

Discussion:

Sickle cell disease: It is an autosomal recessive disease with mutant sickle cell hemoglobin(HbS)

Presentation:
     -Chronic haemolytic anaemia
     -Recurrent painful episodes
     -Acute and chronic organ dysfunction

Precipating factors:
      -Infection
      -Dehydration
      -Acidosis
      -Hypoxia

Pathophysiology:
       -Adenine to thymine substitution in the 6th codon of β globin gene
       -Valine to glutamine substitution on surface HbS
       -Abnormal solubility and polymerization of HbS when deoxygenated
       -Sickling of HbS leading to occlusion of microvasculature

Clinical features:
1.    Vaso occlusive crisis: Plugging of small vessels in bones (most common)
        -Acute severe bone pain (Femur, Humerus, Pelvis, ribs)

2.   Sickle chest syndrome: Common cause of death in adults
        -Followed by VOC
        -Bone marrow infarction
        -Fat emboli to lungs
        -Ventilatory failure

3.  Sequestration crisis:
       -Thrombosis of venous outflow from organ
       -Loss of function and acute painful enlargement
       -Sequestration

4.  Aplastic crisis:
     -Severe self limiting red cell aplasia
     -Anaemia
     -Causes heart failure





Investigations:

-CBC (Reticulocyte count)
-Peripheral blood smear (Sickled erythrocytes)
-Sickling test with sodium metabisulphite
-Hb electrophoresis
-DNA testing for mutations
-LDH
-X Ray (areas of infarction in bones)
-MRI (AVN of femoral and humeral heads)
-USG abdomen (organomegaly)

Treatment:
-Inj. Morphine infusion
-Inj Dynapar
-IV Fluids (N.S)
-100% O2
-Hydroxyurea 500 mg OD
-T. Folic acid 5mg OD
-T. Domstal 10mg TDS

References:
1. BMJ: Article by Davis SC
2. Textbook of Medicine ( Kumar and Clarke)
3. Davidson’s Principles and practice of medicine
4. Cecil-Textbook of medicine
5. Kelley’s Textbook of Rheumatology by Edward D Harrison