What is the best course of action for a female patient with myeloproliferative disease (MPD) presenting with severe left hypochondrial pain radiating to the shoulder and a normal blood film?

From the Guidelines

The best course of action for a female patient with myeloproliferative disease (MPD) presenting with severe left hypochondrial pain radiating to the shoulder and a normal blood film is to treat the underlying cause of the pain, which may be related to splenomegaly or other complications of MPD.

Key Considerations

• The patient's symptoms suggest possible splenomegaly or other abdominal complications related to MPD, which require prompt evaluation and management.
• Aspirin is the first-line systemic therapy for patients with MPD, particularly those with erythromelalgia or other thrombotic complications 1.
• A multidisciplinary team approach is beneficial in the care of patients with MPD, including collaboration with a hematologist, especially for patients with underlying myeloproliferative disease 1.

Management Options

• Cytoreduction therapy may be considered for high-risk patients with MPD, using hydroxyurea or interferon alpha 1.
• Phlebotomy may be necessary to control hematocrit levels in patients with polycythemia vera (PV) 1.
• Pain management should be tailored to the individual patient's needs, and may involve referral to a comprehensive pain rehabilitation center for severe or refractory pain 1.

Important Considerations

• Risk stratification is crucial in managing patients with MPD, and should be based on factors such as age, history of thrombosis, and disease-specific risk scores 1.
• Pregnancy poses unique challenges for patients with MPD, and requires careful management to minimize fetal and maternal complications 1.

From the Research

Presentation and Diagnosis

• The patient presents with severe left hypochondrial pain radiating to the shoulder and a normal blood film, which could be indicative of a splenic issue, such as torsion of a wandering spleen, as seen in a case report 2.
• Myeloproliferative diseases (MPD) can cause splenomegaly, which may lead to symptoms such as abdominal pain and discomfort 3.
• The patient's symptoms and medical history should be thoroughly evaluated to determine the underlying cause of the pain and to rule out other potential causes.

Treatment Options

• Splenectomy may be considered as a treatment option for patients with MPD and symptomatic splenomegaly or hypersplenism, as it can help alleviate symptoms and improve blood counts 3.
• However, the decision to perform a splenectomy should be made on a case-by-case basis, taking into account the patient's overall health, disease severity, and potential risks and benefits of the procedure.
• Other treatment options, such as Janus kinase inhibitors or interferon formulations, may also be considered for patients with early myelofibrosis or other MPDs 4.

Management of MPD in Female Patients

• Female patients with MPD may face unique challenges, such as reduced fertility and increased risk of thrombotic or bleeding complications during pregnancy 5.
• Aspirin may be used to prevent thrombosis in patients with MPD, including those who are pregnant or planning to become pregnant 5.
• The management of MPD in female patients should be individualized, taking into account their reproductive plans, disease severity, and overall health.

Importance of Accurate Diagnosis

• Accurate diagnosis of MPD is crucial to determine the best course of treatment and to prevent potential complications, such as thrombotic events or bleeding disorders 6.
• A thorough evaluation, including blood tests, imaging studies, and bone marrow examination, should be performed to confirm the diagnosis and to rule out other potential causes of the patient's symptoms.