Sickle Cell Crisis in a 25-Year-Old Male: a Case Study

Patient Information

Name: J.T. (initials used for anonymity)

Age: 25 years

Sex: Male

Ethnicity: African American

Medical History: Sickle cell disease (HbSS), multiple previous hospitalizations for vaso-occlusive crises, history of acute chest syndrome, and splenic sequestration crisis as a child.

Medications: Folic acid, acetaminophen for pain management

Chief Complaint

J.T. presented to the emergency department with severe pain in his lower back, chest, and bilateral legs for the past 12 hours. He described the pain as sharp and excruciating, rating it as 9/10 in intensity. He reported experiencing fatigue, fever, and mild shortness of breath. He denied any recent infections but noted that he had not been drinking enough fluids over the past few days.

Initial Vital Signs

Temperature: 101.2°F (38.4°C)

Heart Rate: 112 bpm

Respiratory Rate: 22 breaths per minute

Blood Pressure: 132/86 mmHg

Oxygen Saturation: 95% on room air

Laboratory Findings

Test	Result	Reference Range
Hemoglobin (Hb)	6.8 g/dL	13.5-17.5 g/dL
Hematocrit (Hct)	21.2%	38-50%
Reticulocyte Count	10.5%	0.5-2.5%
White Blood Cell Count (WBC)	15.2 × 10⁹/L	4.0-11.0 × 10⁹/L
Platelets (PLT)	480 × 10⁹/L	150-400 × 10⁹/L
Lactate Dehydrogenase (LDH)	650 U/L	140-280 U/L
Total Bilirubin	4.2 mg/dL	0.1-1.2 mg/dL
Indirect Bilirubin	3.6 mg/dL	0.1-1.0 mg/dL
Serum Ferritin	800 ng/mL	30-400 ng/mL
C-Reactive Protein (CRP)	22 mg/L	<10 mg/L

So... What do the lab findings tell you?

Hemoglobin and hematocrit are low, meaning the patient is losing red blood cells somehow and/or they're not being replaced fast enough. The high bilirubin and LDH points us toward intravascular hemolysis as a mechanism for loss of RBCs.

Now, due to the patient's ethnic background, what we probably want to see next is the RBC morphology of a peripheral blood smear. If sickle cell is not confirmed, we would have to look for other causes of acute hemolysis. A DAT may be ordered to see if antibodies are attaching to RBCs, resulting in some form of autoimmune hemolytic anemia (AIHA).

Peripheral Blood Smear Findings

Presence of numerous sickle cells

Polychromasia (you can see this in the high Reticulocytes above)

Howell-Jolly bodies (suggestive of functional asplenia)

Diagnosis

The patient was diagnosed with an acute sickle cell vaso-occlusive crisis, complicated by hemolysis and possible infection.

Management and Treatment

1. Pain Control:

   • Initiated on intravenous (IV) hydromorphone via patient-controlled analgesia (PCA).

   • Acetaminophen continued for adjunct pain relief.

2. Hydration:

   • IV normal saline (0.9% NaCl) at 125 mL/hr to improve microvascular flow.

3. Oxygen Therapy:

   • Supplemental oxygen via nasal cannula at 2 L/min to maintain oxygen saturation above 95%.

4. Infection Workup and Antibiotics:

   • Blood cultures obtained due to fever.

   • Empiric ceftriaxone started pending culture results.

5. Transfusion Consideration:

   • Due to the patient’s hemoglobin level of 6.8 g/dL and worsening symptoms, a simple blood transfusion was planned.

   • Crossmatching was performed to ensure compatibility with phenotypically matched red blood cells to reduce alloimmunization risk.

6. Continuation of Hydroxyurea:

   • Encouraged to continue hydroxyurea therapy to reduce future crisis episodes.

7. Monitoring:

   • Serial hemoglobin and reticulocyte counts to monitor hemolysis.

   • Daily pain assessments to adjust analgesic regimen as needed.

     
     

Outcome and Follow-up

Over the next 48 hours, J.T. showed improvement in pain and hydration status. Hemoglobin stabilized at 7.2 g/dL after transfusion, and fever resolved without confirmed bacterial infection. He was discharged with instructions to maintain hydration, avoid known triggers (such as cold exposure and dehydration), and follow up with his hematologist for long-term management.

Summary

This case highlights a classic presentation of a sickle cell crisis in a young adult with HbSS disease. The crisis was likely precipitated by dehydration and possible infection. Key laboratory findings included severe anemia, increased reticulocyte count, elevated LDH, and indirect hyperbilirubinemia, all indicative of ongoing hemolysis. Peripheral smear findings confirmed sickle cell presence. Treatment focused on aggressive pain management, IV hydration, oxygen support, and transfusion therapy when indicated. This case emphasizes the importance of early intervention in vaso-occlusive crises to prevent complications such as acute chest syndrome or multi-organ damage. Proper long-term management, including hydroxyurea therapy and hydration strategies, is critical in reducing the frequency and severity of future crises.