Sickle Cell Crises

• Triggers: infection, acidosis, dehydration, cold-exposure, hypoxia, pregnancy
• Presentation: exclude alternative more serious pathology prior to ascribing pain to vaso-occlusive crisis

Effects by Organ System

System	Symptom
CNS	Focal or generalized neurological symptoms, stroke, seizure
Pulmonary	Acute chest syndrome (fever, chest pain, cough, hypoxia, pulmonary infiltrates), pulmonary embolism
GI	Abdominal pain, nausea/vomiting
Renal	Papillary necrosis
GU	Priapism, testicular/ovarian ischemia
Muskuloskeletal	Bone pain (back, proximal extremities), exclude osteomyelitis, avascular necrosis
ID	Infection, functional asplenia (streptococcus, haemophilus)
OB	Preterm labor, placental abruptions, SAB
Ophthalmology	Acute retinal ischemia, hyphema (with intra-ocular hypertension)
Hematology	Sequestration crisis: acute anemia, often post-viral Hemolytic crisis: acute anemia, reticulocytosis, hyperbilirubinemia Megaloblastic crisis: folate deficiency Aplastic crisis: inadequate reticulocytosis

Evaluation

• CBC with reticulocyte count
  •  Hemoglobin: suggests sequestration or hemolytic crisis
  • Reticulocyte index: suggests aplastic or megaloblastic crisis
• LDH/haptoglobin: evaluate for hemolysis
• UA: evaluate for infection/infarction
• CXR: evaluate for acute chest syndrome

Management

• Rehydration (hypotonic fluids)
• Analgesia
• Supplemental oxygen if hypoxic
• Exchange transfusion for priapism, neurologic symptoms, aplastic/sequestration/hemolytic crises

Transfusion Reactions

• Epidemiology: overall 0.25%, 0.09% severe
• Management: stop transfusion

Management by Presumed Etiology

Reaction	Mechanism	Signs/symptoms	Management
Acute, Severe
Acute hemolysis	Incompatibility	Fevers, HR, BP, vomiting, back pain	IVF, vasopressors if needed, furosemide
Anaphylaxis	IgA-mediated	1min: flushing laryngospasm, bronchospasm, BP	Epinephrine, steroids, diphenhydramine, IVF
Sepsis	Bacterial contamination (Y. entercolitica), increased risk in platelet transfusion	Fevers, BP	IVF, vasopressors if needed, broad-spectrum antibiotics
TRALI (transfusion-related acute lung injury)	Non-cardiogenic pulmonary edema, increased risk in FFP transfusion	Hypoxia, respiratory distress, XR bilateral infiltrates	Supplemental oxygen, PPV/ETT
TACO (transfusion-associated circulatory overload)	Hypervolemia in patients with history of CHF	Hypoxia, respiratory distress, heart failure	Supplemental oxygen, PPV/ETT, furosemide
Acute, Minor
Simple febrile reaction	Cytokine-mediated	Isolated fever	Acetaminophen
Minor allergic reaction	Response to transfused plasma proteins	Urticaria, pruritus, flushing	Diphenhydramine
Delayed
Delayed hemolysis	Minor RBC antigens	5-10d, low-grade hemolysis
GVHD	Immunocompromised host	Fever, rash, N/V, transaminitis, pancytopenia
Massive Transfusion
Massive transfusion	Large-volume, refrigerated products	Coagulopathy, hypothermia, hypocalcemia, hyperkalemia, lactic acidosis