Hematology/Oncology

Rapid Review

• Background
  • Disseminated intravascular coagulation is a condition involving abnormal, excessive generation of thrombin and fibrin in the circulating blood, stimulating both coagulation and fibrinolysis in the body. 
  • Most commonly caused by obstetric complications, infection, cancer, and shock
• Signs/Symptoms
  • Excessive bleeding (petechiae, bleeding from venipuncture sites, epistaxis). Predominates in acute DIC
  • Excessive thrombosis (end-organ dysfunction, thrombophlebitis, PE). Predominates in chronic DIC
• Diagnosis
  • Thrombocytopenia, increased PT/PTT, decreased fibrinogen, increased D-dimer. 
• Treatment
  • Treat underlying illness/injury
  • Replacement therapy (fresh frozen plasma, cryoprecipitate, platelets). Consider heparin for chronic DIC where thrombosis predominates.
• Disposition
  • All patients with DIC will generally required admission, likely to the ICU depending on the degree of end-organ damage.

Pearls

• DIC is always a secondary process. Be vigilant in finding the underlying cause (ex. sepsis, leukemia, trauma, liver failure, obstetrics complicaitons, etc.)
• The International Society on Thrombosis and Haemostastis (ISTH) scoring system can help solidify the diagnosis of DIC.

Deep Dive

• Disseminated Intravascular Coagulation (Merck Manual)

Rapid Review

• Background
  • Immunoglobulin A–associated vasculitis (formerly called Henoch-Schönlein purpura) is vasculitis that affects primarily small vessels. It occurs most often in children (2-11 years)
• Signs/Symptoms
  • “Classic Triad” (palpable purpura, arthralgias, and abdominal pain)
  • Commonly causes glomerulonephritis and GI bleeding
• Diagnosis
  • Usually clinical diagnosis
  • Biopsy of affected organ may help confirm diagnosis
• Treatment
  • Mostly supportive. 
  • Arthralgias can be managed with NSAIDs. Prednisone may be useful for abdominal pain and renal disease. Consult nephrology if considering immunosuppressant therapy.
• Disposition
  • Most patients can be discharged home w/ PCP follow up if no CNS findings, significant renal injury, or evidence of of severe GI pathology (ex. intussusception).

Pearls

• Approximately 1/3 of children diagnosed with HSP will relapse within 1 year. These patients will need to be followed closely by their pediatrician for repeat labs/urinalysis.
• Although HSP predominantly affects school aged children, it can be diagnosed in adults as well. These cases tend to cause more renal failure. 

Deep Dive

• Henoch Schonlein Purpura (NUEM Blog)

Rapid Review

• Background
  • A sickle cell crisis is an acutely painful condition that occurs suddenly in patients with sickle cell anemia when sickled red blood cells cause vaso-occlusion, leading to organ ischemia, pain crisis, and other complications. Acute chest syndrome is the most common presentation for adults. 
• Signs/Symptoms
  • Varies based on the site of vaso-occlusion (chest, back, arms, legs, abdomen). 
  • Acute chest syndrome may present with hypoxia, chest pain, fever.
• Diagnosis
  • Clinical diagnosis
  • CXR may show pulmonary infiltrate
• Treatment
  • Analgesia (opiates preferred), hydration, oxygen
  • Evaluate and treat infections
• Disposition
  • Admission is needed for patients with acute chest syndrome, infection, aplastic crisis, or refractory pain. ICU is required if hemodynamically unstable.
  • Discharge is appropriate for patients with a simple pain crisis that has been resolved in the ED. These patients should follow up with their PCP/hematologist.

Pearls

• Many patients will have a individualized prescription/monitoring protocol for vaso-occlusive crises written by their primary sickle cell disease provider. Be sure to use these when available. 
• It is important to not be distracted by the patient’s history of sicke cell disease. Vaso-occlusive crisis should be a diagnosis of exclusion have ruling out other dangerous etiologies (osteomyelitis, septic joint, organ sequestration, intra-abdominal infection, etc.)

Deep Dive

• The Sickle Cell Patient – ED Management of Acute Complications (emDOCs)

Rapid Review

• Background
  • Tumor lysis syndrome is an oncological emergency that occurs when tumor cells break down releasing large amounts of cellular contents into the bloodstream.  Most commonly occurs after initiating chemotherapy of large cancers as tumor cells are lysed. 
  • Characterized by hyperkalemia, hyperphosphatemia, hyperuricemia, and hypocalcemia. 
• Signs/Symptoms
  • Signs/symptoms are vague and non-specific, generally related to electrolyte abnormalities (nausea/vomiting, diarrhea, lethargy, hematuria, arrhythmias, cramps)
• Diagnosis
  • Lab diagnosis requires at least 2 of the following (hyperkalemia, hyperphosphatemia, hyperuricemia, hypocalcemia)
  • Clinical diagnosis = lab diagnosis + 1 clinical manifestation (AKI, seizure, arrhythmia, sudden death)
• Treatment
  • Aggressive hydration. Treat underlying electrolyte abnormalities
  • Consult hematology/oncology
• Disposition
  • Confirmed tumor lysis syndrome usually warrants ICU admission

Pearls

• IV fluids are important for the treatment of tumor lysis syndrome, but be careful not to cause complications of volume overload (ex. pulmonary edema)
• Although tumor lysis syndrome is most frequently occurs in patients undergoing chemotherapy, it can also be the result of hematologic malignancy. 

Deep Dive

• Tumor Lysis Syndrome (Core EM)