Myeloproliferative neoplasms (MPNs) constitute a group of hematologic clonal diseases that affect one or more myeloid lineages with abnormal proliferation. It is rare disease entity and incidence is about 1.15 to 4.99/100 000 person-years among hematological neoplasms for all subtypes of MPNs combined. Patients who present with hepatosplenomegaly, hyperleukocytosis with monocytosis should have routine tests along with bone marrow morphology possibly biopsy, quantiferon TB Gold in tube test, Dengue fever IgM, IgG, NS-1 antigen, cytogenetics t(9;22), BCR cABL fusion gene, JAK-2 V617F, MPL mutations, CALR gene test done along with karyotyping and flowcytometry to evaluate and establish diagnosis towards management.

A patient initially suspected of having a giant cell granuloma was subsequently found-through additional imaging with 99m Tc-sestamibi and ultrasound-to have osteolytic brown tumors caused by a parathyroid adenoma. Brown tumors that relate to primary hyperparathyroidism may mimic other skeletal tumors that contain giant cells, presenting difficulty with accurate diagnosis. Correlative imaging can have a valuable role in such cases, potentially avoiding the extensive work-up usually done for suspected bone metastases. Wepresentacaseofayoungwomanwi th an osteolytic femur and maxillary brown tumors from primary hyperparathyroidism who was misdiagnosed as having giant cell-rich tumors including aneurysmal bone cyst and giant cell granuloma. Correlation between biochemical and multimodality imaging findings eventually led to the accurate diagnosis of brown tumors. CASE REPORTA 23-y-old woman presented with a pathologic fracture of the right femoral neck secondary to osteolytic lesions seen on CT (Fig. 1A). The fracture required hip hemiarthroplasty. On pathologic examination, an aneurysmal bone cyst was found to correlate with the CT findings. The patient's work-up included a skeletal survey that was notable for hypercalcemia and multiple osteolytic lesions, several of which were hot on a whole-body bone scan obtained 3 h after intravenous injection of 740 MBq (20 mCi) of 99m Tcmethylene diphosphonate (Fig. 1B). Contrast-enhanced maxillofacial CT (iopamidol [Isovue; Bracco], 85 mL; 120 kVp; 160 mA; 180-mm field of view) was also performed for evaluation of a left mandibular mass and numbness and revealed multiple osteolytic facial lesions ( Figs. 2A and 2B). Biopsy of the left mandibular mass was consistent with a giant cell granuloma. Further work-up demonstrated an elevated level of serum parathyroid hormone (1,492 pg/mL; reference range, 10-90 pg/mL) and calcium (14.9 mg/dL; reference range, 8.4-10.2 mg/dL), consistent with primary hyperparathyroidism. These findings led to additional imaging, including a parathyroid scan with early (15 min) and delayed (2 h) planar images obtained after intravenous injection of 740 MBq (20 mCi) of 99m Tc-sestamibi (Fig. 3A) and neck ultrasound (Fig. 3B), both of which showed a left inferior parathyroid adenoma, which was later confirmed on pathologic examination after surgical excision. The right hip, left mandible, and numerous other osteolytic skeletal lesions were thus attributed to primary hyperparathyroidism-related multifocal brown tumors. After left inferior parathyroidectomy, the levels of serum parathyroid hormone (32 pg/mL) and calcium (9.3 mg/dL) returned to normal, indicating cure. Follow-up maxillofacial CT revealed regression and sclerosis of the various osteolytic facial lesions (Fig. 2C) after correction of hyperparathyroidism. DISCUSSIONGiant cells can be seen in many bone tumors, including giant cell tumors, aneurysmal bone cysts, giant cell granulomas, giant cell-rich osteosarcomas, chondrosarcomas, and brown tumors associated with hyperparathyroidism (1). Beca...

Source: Liu E, Lee HS, Aronsson CA, et al. Risk of pediatric celiac disease according to HLA haplotype and country. N Engl J Med.