Haemophilia early arthropathy detection with ultrasound and haemophilia joint health score in the moderate haemophilia (MoHem) study

Abstract

Introduction Detection of early arthropathy is crucial for the management of haemophilia, but data on moderate haemophilia are limited. Therefore, we evaluated joint health and treatment modalities in Nordic patients with moderate haemophilia A (MHA) and B (MHB). Aim To explore and compare the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD‐US) and Haemophilia Joint Health Score (HJHS) to detect early arthropathy in moderate haemophilia. Methods A cross‐sectional, multicentre study covering Nordic patients with MHA and MHB. Arthropathy was evaluated by HEAD‐US and HJHS 2.1. Results We assessed 693 joints in 118 patients. HEAD‐US scores (medians [interquartile ranges]) were as follows: elbows 0 points (0–0), knees 0 (0–0) and ankles 0 (0–1). Respectively, by HJHS: elbows 0 (0–1), knees 0 (0–1) and ankles 0 (0–1). Cartilage (14%) and bone (13%) were most commonly affected by HEAD‐US. Frequent HJHS findings were crepitus on motion in knees (39%), and loss of flexion (23%) and extension (13%) in ankles. HEAD‐US correlated strongly with HJHS (elbows r = .70, knees r = .60 and ankles r = .65), but 24% had discordant scores. Joints with HJHS zero points, 5% captured HEAD‐US ≥1 point. Moreover, 26% had HJHS findings without HEAD‐US pathology. Notably, 31% of knees had crepitus on motion and normal HEAD‐US. Conclusion Overall, the joints attained low scores implying good joint health. HEAD‐US correlated strongly with HJHS. In 5%, HEAD‐US detected subclinical pathology. Crepitus on motion was frequently reported despite normal HEAD‐US, thus not necessarily reflecting arthropathy. HEAD‐US therefore improves the joint assessment in moderate haemophilia.

Joint status of patients with nonsevere hemophilia A

“…In addition, a higher proportion of their study population received prophylaxis (38% vs. 16%), which may have contributed to the lower scores observed. Similar to their study, the most frequent findings were crepitus on motion and flexion and extension loss 25 . Other recent work detected arthropathy in 37% of patients with mild hemophilia using HJHS and ultrasound, in which age and baseline factor levels were reported as risk factors for presence of arthropathy 26 …”

The factor VIII treatment history of non‐severe hemophilia A—Response from original authors Abdi et al

“…Positive scores (≥1) on joint level were seen in 19% of the elbows, 20% of the knees, and 23% of the ankles, with corresponding median scores of 0 (IQR 0, 0) for the elbows, 0 (IQR 0, 0) for the knees, and 0 (IQR 0, 1) for the ankles. 3 The difference in observed proportions of joint abnormalities between the patients with moderate hemophilia in the MoHem study and the Spanish cohort may be explained by the difference in age because the patients in the MoHem study were younger than in the Spanish cohort (median age 28 years vs. 51 years, respectively).…”

“…• When should we implement second-line therapy in refractoriness under caplacizumab? Refractory iTTP is currently defined by the lack of a hematological response to standard plasma exchange therapy (PEX), but data about plasma volume (PV) and the number of sessions as standard of care were not given in detail in the paper by Schofield et al 3 There is limited information or consensus available on the management of relapsed/refractory iTTP: PEX may be intensified to 1.5 PV, even twice-daily PEX and high-dose methylprednisolone can be the choice of treatment. 1 Manuscript handled by: David Lillicrap Final decision: David Lillicrap, 01 August 2021…”

“…For example, uncontrolled hypertension more than doubles the risk of intracranial hemorrhage. 5 Because information on these risk factors/comorbidities were not shared in the paper, 3 we do not know whether any contributing factors were present that might have played a role in the bleeding events observed in these four patients.…”

“…The first and second cases experienced progressive pituitary hemorrhage and acute subarachnoid hemorrhage, respectively, at the time of presentation. 3 It is controversial to choose caplacizumab, which is relatively contraindicated in actively bleeding patients according to its product summary. In the second case, caplacizumab was delayed due to the high-risk of hemorrhagic transformation of ischemic lesion, in spite the fact that there is not sufficient data regarding the safety of its use in patients with active or with a high possibility of bleeding.…”