CBL 14 Flashcards
A 40-year-old Jamaican man is admitted with a history of pain over both shoulders, lower back and both thighs over 48 hours duration. The patient has had several hospital admissions with similar presentations. He also mentions being short of breath for the last 24 hours.
Q1. What other information may be helpful in this patient’s history?
Joint & Pain - SOCRATES
SOB - Resp history (ODPARA)
Back pain: MSK?
Previous hospital admissions
PMH / FH; autoimmune diseases
What are lower back pain red flag signs? [+]
Lower back pain red flags:
- Cauda equina syndrome: saddle parasethesia
- PMH of malignancy
- Unexplained weight loss
- loss of anal sphincter tone
- major motor weakness in lower extremities
- fever
- vertebral tenderness
- limited spinal range of motion
- neurological findings persisting for more than one month
- > 50
- palpable pulsatile abdominal mass ? possible abdominal aortic aneurysm
A 40-year-old Jamaican man is admitted with a history of pain over both shoulders, lower back and both thighs over 48 hours duration. The patient has had several hospital admissions with similar presentations. He also mentions being short of breath for the last 24 hours.
Q2. What are the differential diagnoses you may consider based on the given
history alone?
- MI
- HF
- PAD
- SCA
- MSK problems
- Polymyalgia rheumatica
- Acute polyarticular arthritis
- Osteoporosis
- Septic arthritis
- Myeloma
- Autoimmune (SLE / ankylosing spondylitis)
Q3. How would the history help to differentiate the causes of joint pains?
Resp viral illness:
- SOB
- Myalgia
- ? multiple admissions
RA
- pain is reduced with movement, worse at the day
- middle aged
- multiple joints
Anky spond
- Male
- Lower back pain
Sickle cell crisis:
- Ethnicity
- Multiple admissions
- Widespread pain: ?vaso-occlusive crisis
- SOB could be acute chest syndrome
Q4. Below are the examination findings for this patient. How would you interpret these findings
Low Hb with normal MCV:
- normocytic anaemia
High reticulocyte % & raised biliruibin
- High RBC turnover and turnover due to haemolysis
Blood film - target cells:
- Indicates hyposplenism resulting from recurrent splenic infaction
(v slightly raised) WCC & CRP:
- Inflammation
Increased urea
- dehydration
Increased LDH
- may be to haemolysis or hypoperfusion and ischaemia of tissue
Why does this patient have an ejection systolic murmur? [1]
A functional murmur because of disturbed blood flow?
Q6. What is the likely diagnosis?
Acute vaso-occlusive sickle cell crisis with acute chest syndrome
Q7. What other specific examination findings you may expect to find on this patient (from an acute setting)
Splenic sequestration:
- Presents with rapidly enlarging spleen, features of anaemia +/- hypovolaemic shock
Hepatomegaly:
Acute chest syndrome
- acute chest syndrome presents with fever, chest pain, cough, dyspnoea, and hypoxia.
Vaso-occlusive crisss:
- Acute painful crisis
- MI
- Stroke
- VTE
- Renal infarction
- Bone infraction
- Dactylitis
High temperature
Aplastic crisis:
- transient arrest of erythropoiesis, usually induced by infection (e.g. parvovirus B19); causes severe anaemia
- Evidence of erythropoiesis recovery is usually seen after 2-14 days through increase in reticulocyte formation.
Haemolytic crisis:
- Accelerated destruction of both normal and sickled erythrocytes can lead to jaundice and worsening anaemia.
Priapism:
- Obstruction of venous outflow from the corpora cavernosa by sickled cells may cause persistent penile erection accompanied by pain.
Q7. What other specific examination findings you may expect to find on this patient (from a chronic setting) [+]
Anaemia:
- chronic haemolytic anaemia is a constant feature in sickle-cell patients, characterized by pallor, fatigue, and exertional dyspnoea
Infections:
- Functional asplenia due to recurrent splenic infarctions increases susceptibility to encapsulated bacterial infections, such as Streptococcus pneumoniae and Haemophilus influenzae.
Leg ulcers:
- Chronic venous insufficiency resulting from vaso-occlusion can lead to non-healing leg ulcers, predominantly around the medial malleoli.
Ocular complications:
- Retinal vessel occlusions may cause proliferative retinopathy, vitreous haemorrhage or retinal detachment leading to vision loss.
Skeletal complications:
- Chronic bone infarcts can result in avascular necrosis of the femoral and humeral heads.
- Additionally, marrow hyperplasia may cause osteopenia and pathologic fractures.
Renal complications:
- Repeated renal medullary ischemia predisposes patients to papillary necrosis and renal tubular dysfunction.
- This can manifest as nocturia, polyuria, proteinuria or even chronic kidney disease.
Cardiopulmonary complications:
- Chronic anaemia leads to compensatory mechanisms that increase cardiac output. Over time this results in pulmonary hypertension and eventually right-sided heart failure (cor pulmonale).
Neurological complications:
- Cerebral vasculopathy caused by occlusion of cerebral vessels may lead to ischemic or hemorrhagic strokes.
- Silent cerebral infarcts are also common findings on neuroimaging studies.
Growth retardation and delayed puberty:
- Children with sickle-cell anaemia often exhibit growth retardation due to chronic hypoxia and undernutrition related to increased metabolic demands from their condition.
Following is the CXR and what does it show?
Lobar consolidation – lobar pneumonia which may have precipitated the crisis.
OR this is an acute chest syndrome which has been caused by the sickling.
Q10. Are there any other investigations indicated now? [3]
Haptoglobin levels:
- Low in haemolytic anaemias
Group & Save
- patient may need transfusion / cross match
Gel electrophoresis:
- High levels of HbS.
ECHO
- due to murmur
Cultures - blood and sputum
Consider leg USS if worried about PE/DVT
Q11. What are the main priorities of management?
1ST LINE –
oxygen + incentive spirometry
PLUS –
analgesia - subcut morphine
PLUS –
broad-spectrum antibiotics
CONSIDER –
antihistamine (analgesia can give a rash; blood transfusion can give
CONSIDER –
blood transfusion
CONSIDER –
hydration
acute chest syndrome to pneumonia
Treat how you would treat a pneumonia
Describe the management of chronic
1ST LINE –
supportive care + prevention of complications
CONSIDER –
hydroxycarbamide
CONSIDER –
L-glutamine
CONSIDER –
crizanlizumab
CONSIDER –
voxelotor
CONSIDER –
repeated blood transfusions
**2ND LINE – **
haematopoietic stem cell transplantation: curative
What can you give if someone is intolerant to hydroxyurea? [1]
L’glutamine
