Abstract

Background: Scant data exists regarding the burden of fatigue and constitutional symptoms in patients with myeloproliferative disorders (MPDs) (essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis with myeloid metaplasia (MMM)).

Methods: An Internet based survey of MPD patients regarding clinical course, blood counts, constitutional symptoms, and fatigue (FACT-AN and Brief Fatigue Inventory (BFI)).

Results:

Patient Demographics: A total of 1179 MPD patients (median age 56; 41.4% male) internationally (76% USA: 50 states, 30 countries, 6 continents) responded to the survey. Self reported diagnoses were PV (35%), ET (26%), or MMM (39%).

Clinical History: 1025 patients (87%) had undergone MPD therapy (drugs 70.5%, phlebotomy 44.1%, other 16%). Thrombosis (n=261, 22%), hemorrhage (N=272; 23%), and splenomegaly (N=478 (43%) were frequently reported. Blood tests (available in 917) showed 39% were anemic, 7% transfusion dependent.

Symptoms: Fatigue, pruritus, night sweats and bone pain were present across the majority of MPD patients (see table). Additionally it was observed that these symptoms appeared with nearly equal frequency in PV as in MMM patients. However MMM patients displayed more advanced features (fevers, night sweats and weight loss).

MPD Associated Constitutional Symptoms in 1179 Patients

SYMPTOMPV (N=405)ET (N=304)MMM (N=456)MISSING (N=14)TOTAL (N=1179)P VALUE
Fatigue 344 (84.9%) 220 (72.4%) 381 (83.6%) 945 (81.1%) <0.0001 
Pruritus 265 (65.4%) 120 (39.5%) 228 (50.0%) 613 (52.6%) <0.0001 
Night Sweats 199 (49.1%) 123 (40.5%) 254 (55.7%) 576 (49.4%) 0.0002 
Bone Pain 174 (43.0%) 125 (41.1%) 214 (46.9%) 513 (44%) 0.2480 
Fevers 53 (13.1%) 26 (8.6%) 81 (17.8%) 160 (13.7%) 0.0013 
Weight Loss 39 (9.6%) 22 (7.2%) 93 (20.4%) 154 (13.2%) <0.0001 
Spleen Pain 17 (4.2%) 27 (8.9%) 30 (6.6%) 74 (6.3%) <0.0001 
SYMPTOMPV (N=405)ET (N=304)MMM (N=456)MISSING (N=14)TOTAL (N=1179)P VALUE
Fatigue 344 (84.9%) 220 (72.4%) 381 (83.6%) 945 (81.1%) <0.0001 
Pruritus 265 (65.4%) 120 (39.5%) 228 (50.0%) 613 (52.6%) <0.0001 
Night Sweats 199 (49.1%) 123 (40.5%) 254 (55.7%) 576 (49.4%) 0.0002 
Bone Pain 174 (43.0%) 125 (41.1%) 214 (46.9%) 513 (44%) 0.2480 
Fevers 53 (13.1%) 26 (8.6%) 81 (17.8%) 160 (13.7%) 0.0013 
Weight Loss 39 (9.6%) 22 (7.2%) 93 (20.4%) 154 (13.2%) <0.0001 
Spleen Pain 17 (4.2%) 27 (8.9%) 30 (6.6%) 74 (6.3%) <0.0001 

Fatigue (82% MPD linked) was significantly increased across all MPD subtypes compared to published controls for both the BFI (P<0.0001) and Fact-An (P<0.0001). Additionally 69% curtail social activity because of fatigue, 35% need assistance with activities of daily living, and 11% reported MPD associated medical disability. Fatigue strongly correlated with multiple MPD disease features including: anemia, bone pain, constitutional symptoms, pruritus, splenic pain, smoking, thrombosis, and hemorrhagic events (all p<0.0001). Interestingly, even in “asymptomatic” MPD patients (those without anemia, splenomegaly, or thrombohemorrhagic complications) (n=279)) 24% (41% PV, 31% ET, 28% MMM) described significant fatigue worse than published controls.

Conclusions: Fatigue and other constitutional symptoms are a significant burden to MPD patients, despite current therapies employed. Additionally fatigue is also a significant problem even in MPD patients lacking other advanced disease features. Clearly, direct attempts to improve fatigue (through pharmacologic and non-pharmacologic interventions) are necessary.

Disclosure: No relevant conflicts of interest to declare.

Author notes

*

Corresponding author