A case of refractory hypercalcemia. Elusive diagnosis and difficult management
B Inayat, A Iqbal, N Haya, Great Western Hospital NHS Trust Foundation Swindon
Para-neoplastic Hypercalcemia usually presents with markedly elevated serum calcium levels. Several potential mechanisms leading to the development of hypercalcemia of malignancy have been described, including parathyroid hormone-related peptide-mediated humoral hypercalcemia, osteolytic metastasis-related hypercalcemia, 1, 25 Vitamin D-mediated hypercalcemia, and parathyroid hormone mediated hypercalcemia. In addition to detailed history and physical examination, markers for above mentioned processes should be measured.
We present a case of refractory hypercalcemia in an 85 years old, Caucasian man, with background of myelodysplastic syndrome, pancytopenia, splenomegaly and renal impairment. He initially presented to acute admissions, through his GP, with new onset, severe hypercalcemia and worsening renal impairment. Physical examination was unremarkable. Medications included Colecalciferol, Ganfort, Paracetamol. Laboratory investigations showed Calcium 3.44mmol/L (2.12-2.62), PTH <0.6pmol/L (1.8-6.8), Vitamin D 39nmol/L. Myeloma screen was negative, B2 microglobulin was high. CT abdomen and pelvis showed enlarged spleen, T6 vertebral collapse. Bone marrow biopsy showed no evidence of myeloma and JAK2 mutation negative. Bone scan showed normal uptake. 1,25 dihydroxyvitamin D was 134pmol/L (55-139). Subsequent PET scan showed splenomegaly with increased uptake.
Above pattern of Lab results confirm a process of PTH independent hypercalcemia. He was resuscitated with intravenous fluids, renal dose pamidronate, and calcitonin. Regular Laboratory monitoring was arranged. Subsequently, zolendronate was given due to refractory hypercalcemia. Hematologist did not consider Myelodysplasia to be the likely cause of severe hypercalcemia. A possible presence of intra-splenic lymphoma has been raised.
There is continuous MDT involvement including Endocrinologist, Haematologist and radiologist. Tertiary centre-based haematology MDT suggested spleen biopsy or splenectomy if biopsy not possible. Interventional radiologist is concerned about increased risk of bleeding from spleen biopsy given age, frailty and co-morbidities.
Patient declined spleen biopsy. MDT planned to start trial of empirical steroids, and to study blood film for flow cytometry.
This case clearly illustrates importance of MDT role in the management of this complex case where diagnosis still remain elusive and optimal management challenging.