What is the appropriate management for a female patient with myeloproliferative disease (myeloproliferative neoplasm) presenting with severe left upper quadrant abdominal pain radiating to the shoulder and a normal peripheral blood smear?

From the Guidelines

I would recommend conservative management (option D) for this female patient with myeloproliferative disease presenting with severe left hypochondrial pain referred to the shoulder and a normal blood film. The clinical presentation suggests splenic infarction, a known complication of myeloproliferative disorders, but with a normal blood film and no evidence of splenic rupture, immediate surgical intervention is not warranted. Conservative management should include:

• Adequate pain control with analgesics (starting with NSAIDs like ibuprofen 400-600mg every 6-8 hours, progressing to opioids if needed)
• Intravenous hydration
• Close monitoring of vital signs and hemoglobin levels. The patient should undergo further imaging studies such as an abdominal CT scan with contrast to confirm splenic infarction and rule out other complications, as recommended by the European LeukemiaNet 1. Most splenic infarcts resolve spontaneously with supportive care. Surgical interventions like splenectomy (options A and B) or endovascular stenting (option C) would be reserved for complications such as splenic rupture, abscess formation, pseudocyst development, or persistent symptoms despite conservative management. The underlying myeloproliferative disorder should also be addressed with appropriate hematology consultation and disease-specific therapy to prevent recurrent thrombotic events, following the guidelines outlined by the NCCN 1. It is essential to assess the symptom burden using tools like the Myeloproliferative Neoplasm Symptom Assessment Form (MPN-SAF) to monitor symptom status during treatment, as suggested by the NCCN clinical practice guidelines in oncology 1. By prioritizing conservative management and addressing the underlying condition, we can minimize the risk of morbidity, mortality, and improve the patient's quality of life.

From the Research

Diagnostic Approach

• The patient's symptoms of severe left hypochondrial pain referred to the shoulder, along with a history of myeloproliferative disease, suggest the possibility of splenic infarction 2, 3, 4.
• A normal blood film does not rule out splenic infarction, as this condition can occur in patients with hematologic disorders, including myeloproliferative diseases 4.
• Diagnostic tests such as CT scan, ECG, echocardiography, and blood cultures are recommended to evaluate the patient's condition 3.

Management Options

• Conservative treatment may be adequate for managing symptoms of splenic infarction in patients with myeloproliferative diseases, as seen in a study where no serious complications were noted in patients with splenic infarctions 4.
• Splenectomy may be considered in select patients with massive splenomegaly and related symptoms, but it is associated with substantial morbidity and mortality 5, 6.
• Other management options, such as JAK 1/2 inhibitors, splenic irradiation, and partial splenic artery embolization, may be considered in patients with myelofibrosis and symptomatic splenomegaly 5.

Considerations

• The patient's underlying myeloproliferative disease and the presence of splenomegaly should be taken into account when deciding on the best course of management 4, 5, 6.
• The risk of thrombosis and other complications should be considered when evaluating the patient's condition and deciding on treatment options 4.