Subjective
Date of visit
Reason for visit (follow-up for PMF, evaluation of new symptoms)
Current symptoms:
Constitutional symptoms (common):
Fatigue (often the most bothersome symptom)
Night sweats
Weight loss
Splenomegaly symptoms (enlarged spleen):
Early satiety (feeling full quickly)
Left upper quadrant abdominal pain or discomfort
Difficulty lying on the left side
Bone pain (may occur)
Bleeding symptoms (less common):
Easy bruising
Petechiae (red pinprick spots on skin)
Nosebleeds
Progression of existing symptoms since last visit
Objective
Vital signs (temperature, blood pressure, heart rate) – fever may suggest infection
Physical exam:
General (assessing for weight loss, pallor, signs of bleeding)
Abdominal exam (palpating for splenomegaly)
Lymph node exam (assessing for lymphadenopathy)
Laboratory Tests
Complete blood count (CBC):
Anemia (low red blood cell count)
Thrombocytosis (high platelet count) or thrombocytopenia (low platelet count) – either can occur in PMF
Leukopenia (low white blood cell count) – may be present
Peripheral blood smear (to examine blood cell morphology for abnormalities)
Bone marrow biopsy and aspirate (definitive test for PMF) – may show characteristic findings of fibrosis
Serum lactate dehydrogenase (LDH): often elevated in PMF
Serum uric acid: may be elevated due to increased cell breakdown
Imaging Tests (may be ordered depending on clinical presentation):
Abdominal ultrasound: to assess spleen size and other abdominal organs
Liver ultrasound: to assess for liver involvement
Assessment
Primary myelofibrosis confirmed (based on bone marrow biopsy and clinical presentation)
Disease stage (based on specific scoring systems) – helps guide treatment decisions
Severity of symptoms (constitutional, splenomegaly-related)
Presence of cytopenias (anemia, thrombocytopenia, neutropenia) – can affect blood cell production
Evaluation for potential complications (portal hypertension, infections)
Plan
Treatment plan based on disease stage, symptoms, and patient characteristics:
Symptom management: to improve quality of life (pain management, blood product transfusions)
JAK inhibitor therapy: can improve symptoms and reduce spleen size (standard treatment for many PMF patients)
Other medications: to manage specific complications (e.g., antibiotics for infections)
Stem cell transplantation (SCT): curative option for some younger patients with a suitable donor
Referral to a hematologist specializing in myeloproliferative neoplasms (MPN) for complex cases
Education
Explain primary myelofibrosis, a chronic bone marrow disorder characterized by bone marrow fibrosis and abnormal blood cell production.
Discuss the treatment plan, potential side effects, and importance of medication adherence.
Importance of recognizing and reporting new symptoms (infections, bleeding)
Information about the disease course, potential complications, and the need for regular follow-up.
Provide resources for support groups or patient advocacy organizations.
Follow-up
Schedule for follow-up appointments:
Monitor symptoms and response to treatment
Monitor blood counts for cytopenias
Assess for complications
Adjust medication regimen as needed
Consider splenectomy (surgical removal of the spleen) for severe splenomegaly or uncontrollable symptoms (if not a candidate for SCT)
Disclaimer: This information is for educational purposes only and should not be interpreted as medical advice. Please consult with a qualified healthcare professional for diagnosis and treatment of primary myelofibrosis