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Qualityforlife CIC
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    • About GVHD
    • MSK Manifestation in GVHD
    • Assessing AROM in GVHD
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  • Meet the Team
  • Education
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  • More
    • Home
    • About Transplant
      • HCT
      • Get Ready for Transplant
      • Rehabilitation & HCT
    • Graft Versus Host Disease
      • About GVHD
      • MSK Manifestation in GVHD
      • Assessing AROM in GVHD
      • Patient Exercise & Info
    • Meet the Team
    • Education
      • Upcoming Events
    • Post-Symposium Downloads

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  • Home
  • About Transplant
  • Graft Versus Host Disease
  • Meet the Team
  • Education
  • Post-Symposium Downloads

MSK MANIFESTATION

Skin & Fascia

Loss of Muscle Strength

Loss of Muscle Strength

image2780

  • Sclerotic-type chronic GVHD (ScGVHD) of the skin encompasses several cutaneous presentations characterized by inflammation and progressive fibrosis of the dermis and subcutaneous tissues, resembling morphea, systemic sclerosis, or eosinophilic fasciitis.
  • One of the other distinctive feature for chronic GVHD is depigmentation.

Loss of Muscle Strength

Loss of Muscle Strength

Loss of Muscle Strength

image2781

 

  • Muscle loss, most frequently a result of disuse, deconditioning, or side effects of immunosuppressive treatment, particularly corticosteroids. 
  • Reduced respiratory and skeletal muscle strength and submaximal exercise capacity in the post-transplant period.


Steroid-induced Myopathy:  

  • Acute - typically occurs within 1 week of high-dose oral corticosteroid use and can be associated with rhabdomyolysis and pain. 

Bone

Loss of Muscle Strength

Inflammatory Myositis

image2782

 

As many as 50% of patients who undergo HSCT develop osteopenia or osteoporosis, and cGVHD is associated with an even higher incidence. This is often the result of chronic glucocorticoid use, a major risk factor for osteopenia and osteoporosis because of increased bone turnover. 


The HSCT itself causes a fundamental alteration of bone mineral metabolism, with loss seen in the first 6 to 12 months. Bone density loss in cGVHD is typically seen more in the femoral heads than the vertebrae, a distinction when compared with menopausal osteoporosis,  though the humeral head, knees, and ankles can also be affected.


Lastly, patients with osteoporosis can develop hip flexor contractures due to sustained forward-flexed posture. A tight iliopsoas muscle may lead to worsening lumbar lordosis, increasing pain, and adding force being applied to vertebrae already prone to fracture. 

Inflammatory Myositis

Inflammatory Myositis

Inflammatory Myositis

image2783

 Muscle mass and strength may be compromised because of an inflammatory myositis, mimicking polymyositis or dermatomyositis, that is a direct, immune-mediated result of cGVHD.   Associated with tapering of immunosuppressant medications and is associated with the same genetic markers seen in patients with this autoimmune disease who have not undergone HSCT. Typically, this presents with painful, symmetric proximal weakness. 

Peripheral Nerves

Inflammatory Myositis

Peripheral Nerves

image2784

cGVHD is associated with several possible neurologic sequelae, including mononeuropathies, generalized peripheral neuropathy, and inflammatory neuropathy. A combination of multiple neuropath. This has also been shown to cause a neuropathic process that resembles acute inflammatory demyelinating polyneuropathy (AIDP), and is thought to be a result of direct infiltration of peripheral nerves by donor T cells.


An EMG can be diagnostic and can show demyelination, axon loss, or both. The first electromyographic sign of AIDP is an absent F-response, with subsequent findings of conduction block and denervation. 


Nerve entrapment can be either by mechanical compression or fascial inflammation. As the inflamed fascia and/or skin surrounding peripheral nerves become fibrosed, the nerve may become entrapped and damaged. 


Nerves at high risk for entrapment are those with little surrounding tissue, such as the ulnar nerve at the cubital tunnel and peroneal nerve at the fibular head. The median nerve at the carpal tunnel can also be damaged due to wrist flexion contractures.

Myasthenia Gravis

Inflammatory Myositis

Peripheral Nerves

image2785

 In rare instances, develops when tapering immunosuppressive medication because of pre-existing autoantibodies against postsynaptic acetylcholine receptors. Most commonly seen in patients who received the HSCT for aplastic anemia. Symptoms typically are progressive weakness with exertion, with recovery of strength after rest. Ptosis is a common first symptom and its presence along with generalized weakness. May warrant an EMG that includes repetitive stimulation studies and blood tests to detect antibodies to acetylcholine receptors [6].  

References:

  •  Rosenthal EA, Ho PS, Joe GO, Mitchell SA, Booher S, Pavletic SZ, Baird K, Cowen EW, Comis LE. Motor ability, function, and health-related quality of life as correlates of symptom burden in patients with sclerotic chronic graft-versus-host disease receiving imatinib mesylate. Supportive Care in Cancer. 2019 Dec 6:1-1. 
  •  Spiesshoefer J, Henke C, Kabitz HJ, Nofer JR, Mohr M, Evers G, Strecker JK, Brix T, Randerath WJ, Herkenrath S, Schmidt LH. Respiratory Muscle and Lung Function in Lung Allograft Recipients: Association with Exercise Intolerance. Respiration. 2020;99(5):398-408. 
  •  Meng L, Ji S, Wang Q, Bu B. Polymyositis as a manifestation of chronic graft‐versus‐host disease after allo‐HSCT. Clinical Case Reports. 2018 Sep;6(9):1723. 
  •  Cho WK, Ahn MB, Lee JW, Chung NG, Jung MH, Cho B, Suh BK. Low bone mineral density in adolescents with leukemia after hematopoietic stem cell transplantation: prolonged steroid therapy for GvHD and endocrinopathy after hematopoietic stem cell transplantation might be major concerns?. Bone Marrow Transplantation. 2017 Jan;52(1):144-6. 
  •  Lin JN, Chen HJ, Yang CH, Lai CH, Lin HH, Chang CS, Liang JA. Risk of osteoporosis and pathologic fractures in cancer patients who underwent hematopoietic stem cell transplantation: a nationwide retrospective cohort study. Oncotarget. 2017 May 23;8(21):34811. 
  •  Meng L, Ji S, Wang Q, Bu B. Polymyositis as a manifestation of chronic graft‐versus‐host disease after allo‐HSCT. Clinical Case Reports. 2018 Sep;6(9):1723. 
  •  Brown VI. Neurologic and Sensory Complications Associated with HSCT. InHematopoietic Stem Cell Transplantation for the Pediatric Hematologist/Oncologist 2018 (pp. 343-361). Springer, Cham. 
  •  Vukić T, Robinson Smith S, Ljubas Kelečić D, Desnica L, Prenc E, Pulanić D, Vrhovac R, Nemet D, Pavletic SZ. Joint and fascial chronic graftvs-host disease: correlations with clinical and laboratory parameters. Croatian medical journal. 2016 Jun 15;57(3):266-75. 
  •  Avila JD, Bucelli R, Varadhachary A. Myasthenia gravis after hematopoietic stem cell transplantation: A comprehensive review (P5. 073). 

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Contact Name: Jaleel Mohammed PT, MSc