Progressive low back pain. Personal medical history revealed intermittent low back pain for several years, but no trauma nor other chronic diseases. On physical examination, a slight kyphosis of the thoracolumbar spine was found. Neurological and laboratory examinations were normal.
A 27-year-old man was admitted to the orthopedic department of the hospital because of progressive low back pain. Personal medical history revealed intermittent low back pain for several years, but no trauma nor other chronic diseases. On physical examination, a slight kyphosis of the thoracolumbar spine was found. Neurological and laboratory examinations were normal.
Langerhans cell histiocytosis or histiocytosis X is an uncommon disease with multisystemic presentation. Pathohistologically, it is characterized by a granulomatous proliferation of the reticulum cells at one or several sites in the reticuloendothelial system. The etiology is unknown. It is possibly related to prior infection. The disease may occur in the skeleton, spleen, thymus, lymph nodes and skin. There are three major manifestations of histiocytosis X: eosinophilic granuloma, Hand-Schüller-Christian disease and Letterer-Siwe disease. It is believed that these three disorders are expression of the same basic pathological process. The role of MRI in the evaluation of Langerhans cell histiocytosis is twofold: identification of the lesion and staging, i.e. definition of location and extent. On MRI the skeletal lesions are often well defined, isointense or hypointense on T1-weighted images and definitely hyperintense compared to bone marrow on fat saturated T2-weighted images. The lumbar and thoracic spine are predominantly affected. Involvement of one or several adjacent vertebrae is often seen. Intervertebral discs usually remain normal, but a paraspinal mass may be evident, simulating a soft-tissue abscess. The imaging findings of skeletal histiocytosis resemble those of osteomyelitis, Ewing’s sarcoma, lymphoma or multiple myeloma. Therefore, these diseases should be considered in the differential diagnosis.
Langerhans cell histiocytosis
1. X-ray Examination:
• Mild kyphotic deformity (mild gibbus) can be observed in the thoracolumbar region on the lumbar and thoracic spine X-rays. The density of the vertebral bodies shows no significant change, but the edges may exhibit slight destruction or morphological abnormalities.
• Adjacent intervertebral spaces are essentially normal, with no evident narrowing or signs of disc destruction.
2. MRI Findings:
• Lesions in the affected vertebral bodies typically appear isointense or slightly hypointense on T1-weighted sequences, while showing notably high signal on T2 or fat-suppressed T2-weighted sequences. This suggests increased water content or histological changes compared to normal bone marrow.
• The lesion may involve multiple adjacent vertebral bodies, though the intervertebral discs often remain intact. In rare cases, a soft tissue mass adjacent to the vertebral bodies may be seen, requiring differentiation from infectious or neoplastic lesions.
• No obvious significant signal abnormality suggests spinal cord compression or nerve root displacement, and neurological examinations reveal no prominent positive findings.
Based on the imaging findings, the patient’s age (27 years old, male), main complaint (progressive lower back pain), and normal physical and laboratory results, the possible diagnoses include:
Taking into account the patient’s age, clinical symptoms (intermittent lower back pain over years, recently worsened), imaging characteristics (localized vertebral lesions, low or isointense signals on T1, high signal on T2, no significant disc destruction), and normal routine blood and neurological examinations, the most likely diagnosis is: Langerhans Cell Histiocytosis (LCH; Histiocytosis X).
For definitive confirmation, a biopsy with histopathological examination, in combination with immunohistochemistry (CD1a or Langerin markers), may be considered.
1. Treatment Strategy:
• Local Treatment: For localized, single-vertebra lesions, lesion curettage with pathological verification can be followed by local radiotherapy or a small dose of local corticosteroid injection based on individual circumstances.
• Systemic Treatment: In cases of multifocal disease or systemic symptoms, chemotherapy, radiotherapy, or systemic corticosteroid therapy may be administered as needed.
• Symptom Management: Includes analgesic medications, physical therapy, and bracing to relieve pain and prevent further structural compromise.
2. Rehabilitation and Exercise Prescription:
• General Principles: Once pain is managed and vertebral stability is ensured, gradually engage in muscle-strengthening exercises and cardiovascular training, avoiding high-impact activities that might cause additional injuries.
• Initial Phase (Pain Relief Phase):
– Focus on low-intensity core stability exercises, such as planks and supine leg raises, holding each movement for 5-10 seconds, resting for 10 seconds, and repeating for 5-8 sets;
– Perform small-range isometric lumbar and back muscle exercises with breathing control, 3-4 times per week, avoiding excessive fatigue.
• Intermediate Phase (Strengthening Phase):
– Increase core muscle training, including bridging exercises, side planks, and dumbbell or resistance band-backed lumbar strengthening, 3-5 times a week for 20-30 minutes each session;
– Gradually introduce flexibility training and moderate-intensity, low-impact aerobic activities (e.g., brisk walking or elliptical training) within pain tolerance, aiming for 20 minutes per session at 50-60% of maximum heart rate.
• Later Phase (Functional Return Phase):
– If bone stability is confirmed, gradually resume normal activities and weight-bearing exercises under the guidance of physicians and rehabilitation specialists;
– Carefully increase the intensity of core and lower back strengthening, adding more functional movement training (e.g., lower limb stability work, balance training), 3-5 times per week, at least 30 minutes per session.
• Continuous Monitoring and Adjustment:
– Adjust the frequency, intensity, time, and progression of training (according to FITT-VP principles) based on clinical changes, imaging follow-up, and pain or fatigue levels;
– Avoid sudden increases in workout volume or excessive loading to prevent reinjury to the vertebrae.
[Disclaimer] This report is based on the existing imaging and clinical information and is intended as a reference only. It should not substitute for in-person consultation with a professional physician. Should any questions or changes in condition arise, please seek specialist advice promptly for further evaluation and treatment.
Langerhans cell histiocytosis