Spontaneous Remission Of Severe Systemic Langerhans Cell Histiocytosis With Bladder Involvement: A Case Study
Magaton, I. M., Tzankov, A., Krasniqi, F., Rottenburger, C., Zanetti-Daellenbach, R., Grendelmeier, P., Heinzelmann-Schwarz, V., Mayears, M., & Schwab, F. D. (2017). spontaneous Remission of Severe Systemic Langerhans Cell Histiocytosis with Bladder Involvement: A Case Study. Case reports in oncology, 10(3), 876–884. https://doi.org/10.1159/000480696
View Original Source →Abstract
<b><i>Background:</i></b> The clinical presentation of Langerhans cell histiocytosis (LCH) is heterogeneous ranging from single-organ involvement to systemic disease causing substantial morbidity and mortality. We describe an unusual course of severe multisystem LCH with spontaneous remission. <b><i>Case Presentation:</i></b> We report on a 45-year-old Caucasian woman with cervical cancer, FIGO stage IVB. Five months after the end of combined radiochemotherapy and brachytherapy, the patient was readmitted because of severe dysuria. Sterile leukocyturia was seen, and cystoscopy revealed only 3 unspecific small mucosal lesions compatible with postradiation cystitis. Incidentally, a computed tomography (CT) scan of the body showed diffuse micronodular and cystic lesions in lungs and hypodense lesions in the liver. Biopsies revealed infiltrations of CD1a and Langerin (CD207)-positive histiocytes in the lung, liver, and bladder. Additionally, positron emission tomography-CT (PET-CT) was compatible with bone involvement. Retrospective analysis revealed that the increase in alkaline phosphatase might have been a surrogate of bone marrow infiltration with osseous activity. Repeated pneumothoraces occurred, and only one course of vinblastine-prednisolone could be applied. Despite ongoing tobacco consumption and without further therapy, PET-CT showed considerable remission 2 months later. However, despite stable remission, documented by serial PET and conventional CT scans, persistent infiltration of the bladder by Langerhans histiocytes could still be demonstrated 17 months later. Unfortunately, cervical cancer recurred and progressed. <b><i>Conclusion:</i></b> Multisystem LCH may rapidly occur, may be oligosymptomatic and, even in high-risk cases, remission without specific therapy might occur. Whether alkaline phosphatase might be a surrogate to monitor osseous disease activity has to be further explored.
Case Details
Disease Location
Lung, liver, bladder, uterus
Personal Characteristics
45-year-old caucasian woman, smoker 45 pack-years, cocaine and heroin, stopped drug abuse a long time ago. History of gastric banding, abdominalominoplasty, and recurrent abscess excision in the breast due to mastitis non-puerperalis.
Clinical Characteristics
Was diagnosed with stage ivb cervical cancer. Obstruction of the right kidney was seen, and a ureter catheter was inserted. The cancer was treated with 6 courses of combined radiochemotherapy followed by brachytherapy. Eight months after cancer diagnosis and 5 months after the end of the radiation-chemotherapy, the patient complained again of heavy dysuria, pollakiuria, and additionally lower-abdominal cramps. Urinary tract infections were assumed empiric therapy with different antibiotics was not successful in pain relief. Diagnosis of overactive bladder syndrome and postradiation irritation was made. Analgesic and spasmolytic therapy were not sufficient to control the complaints. Sterile leukocyturia was seen, and cystoscopy revealed 3 unspecific small mucosal lesions and one ulceration compatible with postradiation cystitis. CT of the thorax and abdomen showed multiple micronodular and cystic lesions in both lungs and diffuse hypodense lesions in the liver. Biopsies of the liver and the lung revealed infiltrations of langerin (CD207) and cd1a-positive histiocytes admixed with eosinophils in both organs, and lch was diagnosed. PET-CT was performed, and the final diagnosis of multisystem lch involving lungs, liver, bone, and the bladder was established
Remission Characteristics
The patient became asymptomatic, and two months later a follow-up PET-CT showed remarkable partial remission of lch
Treatment & Mechanisms
Proposed Remission Mechanisms
Not discussed
Clinical Treatment
Radiochemotherapy, brachytherapy. Oxycodon, morphine, and trospium chloride ureter catheter