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The hallmark of osteopetrosis is generalized increased bone mineral density (BMD). However, the bone is fragile and fractures are common. Autosomal recessive osteopetrosis is usually a severe disorder and often life\u2010threatening in childhood. We present male siblings with autosomal recessive osteopetrosis due to biallelic variants in\n                    <jats:italic>TCIRG1<\/jats:italic>\n                    who survived childhood and underwent hematopoietic stem cell transplant (HSCT) in adulthood. One sibling died of posttransplant complications. After transplant, the other sibling had improvement of multiple clinical parameters, including some decline in BMD\n                    <jats:italic>Z<\/jats:italic>\n                    \u2010scores by dual\u2010energy X\u2010ray absorptiometry (DXA) and cessation of fractures. However, spine quantitative computed tomography 11\u2009years after transplant demonstrated an anvil pattern of sclerosis with BMD\n                    <jats:italic>Z<\/jats:italic>\n                    \u2010score of +18.3. High\u2010resolution peripheral quantitative computed tomography (HR\u2010pQCT) of the tibia demonstrated near complete obliteration of the marrow space combined with an unusual cortical phenotype, suggesting extensive cortical porosity at the distal tibia. This case highlights that despite successful transplantation and subsequent improvement in clinical parameters, this patient continued to have significantly elevated bone density and decreased marrow space. Transplant\u2010associated increased cortical porosity is multifactorial and occurs in two\u2010thirds of non\u2010osteopetrotic patients undergoing HSCT. This finding after transplant in osteopetrosis may suggest particular sensitivity of the cortical bone to resorptive activity of transplanted osteoclasts. The case also suggests HR\u2010pQCT may be a useful modality for imaging and assessing the therapeutic effects on bone in individuals with osteopetrosis. \u00a9 2022 The Authors.\n                    <jats:italic>JBMR Plus<\/jats:italic>\n                    published by Wiley Periodicals LLC on behalf of American Society for Bone and Mineral Research.\n                  <\/jats:p>","DOI":"10.1002\/jbm4.10616","type":"journal-article","created":{"date-parts":[[2022,2,26]],"date-time":"2022-02-26T05:39:32Z","timestamp":1645853972000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Unusual Cortical Phenotype After Hematopoietic Stem Cell Transplantation in a Patient With Osteopetrosis"],"prefix":"10.1093","volume":"6","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0152-7280","authenticated-orcid":false,"given":"Sonia","family":"Afshariyamchlou","sequence":"first","affiliation":[{"name":"Department of Medicine Indiana University School of Medicine  Indianapolis IN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Ng","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Minnesota Medical School  Minneapolis MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Asmaa","family":"Ferdjallah","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Minnesota Medical School  Minneapolis MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6415-4936","authenticated-orcid":false,"given":"Stuart J.","family":"Warden","sequence":"additional","affiliation":[{"name":"Department of Physical Therapy Indiana University School of Health and Human Sciences  Indianapolis IN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Niziolek","sequence":"additional","affiliation":[{"name":"Department of Radiology Indiana University School of Medicine  Indianapolis IN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8914-0610","authenticated-orcid":false,"given":"Imranul","family":"Alam","sequence":"additional","affiliation":[{"name":"Department of Medicine Indiana University School of Medicine  Indianapolis IN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynda E.","family":"Polgreen","sequence":"additional","affiliation":[{"name":"The Lundquist Institute at Harbor\u2010UCLA Medical Center  Torrance CA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7284-3467","authenticated-orcid":false,"given":"Erik A.","family":"Imel","sequence":"additional","affiliation":[{"name":"Department of Medicine Indiana University School of Medicine  Indianapolis IN 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