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Pancytopenia Resulting from Renal Osteodystrophy As a Consequence of Secondary Hyperparathyroidism

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Pancytopenia Resulting from Renal Osteodystrophy As a Consequence of Secondary Hyperparathyroidism Central JSM Clinical Case Reports Case Report *Corresponding author Fatima Meraj, Consultant Haematologist, The Indus Hopsital, The Indus Hospital, Plot C-76, Sector 31/5, Pancytopenia Resulting from Opposite Darussalam Society, Korangi Crossing, Karachi-75190, Pakistan, Tel: +92 (21) 35112709-17; E-mail address: [email protected] Renal Osteodystrophy as A Submitted: 20 October, 2020 Accepted: 26 November, 2020 Consequence of Secondary Published: 28 November, 2020 Copyright © 2020 Meraj F, et al. ISSN: 2373-9819 Hyperparathyroidism OPEN ACCESS 1 1 2 Fatima Meraj *, Sumaira Sheikh , and Hafsa Muhammad Hanif Keywords 1Consultant Haematologist, The Indus Hopsital, Karachi, Pakistan • Renal osteodystrophy 2Resident, Haematology Dept., The Indus Hospital, Karachi, Pakistan • Osteitis fibrosa cystica • End-stage renal disease • Hyperparathyroidism Abstract • Howship’s lacunae Secondary hyperparathyroidism is a known complication of chronic renal disease. Excessive parathyroid hormone induces a form of renal osteodystrophy characterized by bony remodeling (by stimulating osteoblastic and osteoclastic activities) and myelofibrosis. We report the case of a 22-year-old lady with end-stage-renal disease and marked hyperparathyroidism (1140.90pg/ml; reference range: 15-68pg/ml) who developed pancytopenia despite nutritional supplements and weekly erythropoietin injections. Bone marrow examination revealed findings consistent with renal osteodystrophy. INTRODUCTION osteoid [2]. Renal osteodystrophy is a component of the mineral and mineralization, resulting in increased unmineralized bone metabolism disorders that develop as a sequel of CKD (CKD- MBD) [1]. CKD MBD is characterized by abnormalities in calcium, phosphorous, vitamin D and PTH metabolism, abnormalities in boneThe biopsy definitive specimen. diagnosis However, of serum renal PTH osteodystrophy levels can be usedand categorization of subtype are done on a “tetracycline-labelled” patientsbone structure with CKD. and turnover, and soft tissue calcifications. Renal as a surrogate indicator of the various subtypes as follows: PTH osteodystrophy is defined as an alteration in bone morphology in mixedlevels <100uremic pg/mL osteodystrophy are associated [3]. with adynamic bone disease, while PTH >450 pg/mL is suggestive of osteitis fibrosa cystica or RenalOsteitis osteodystrophy fibrosa cysticais classified into four types as follows: is indicated in patients in whom determination of the type of Bone biopsy is the gold standard diagnostic modality. It • osteoclastic activity, as a result characterized of persistent by elevationhigh bone in turnover resulting from increased osteoblastic and PTH [1]. renal osteodystrophy will influence management decisions [4]. characterized by low bone Examples include [5]: Adynamic bone disease for parathyroidectomy due to clinical features of • hyperparathyroidismPatients with end-stage but kidney have indeterminatedisease who are PTH planned levels • osteoclastic activity, as a result of excessive suppression ofturnover PTH production resulting byfrom medication decreased (phosphate osteoblastic binders) and [2]. (<450pg/ml). unexplained bone pains or fractures. Osteomalacia characterized by decreased bone • Patients with chronic kidney disease who have mineralization. In the past this was seen as a complication • aluminium deposition in bone [2]. Since the abandonment • To confirm the diagnosis of adynamic bone disease with tetracycline (usually administered for 3 days, 21 days of aluminium–basedaluminium based phosphate phosphate binders, binders, this complicationresulting in apart).A specimen Tetracycline is obtained binds to fromhydroxyapatite the iliac crest in bone after and labeling emits is now uncommon. Mixed uremic osteodystrophy bone turnover and a disproportionate reduction in bone fluorescence, allowing the identification of bone. The KDIGO • – characterized by high (Kidney Disease: Improving Global Outcomes) working group on renal osteodystrophy has proposed the “TMV” classification Cite this article: Meraj F, Sheikh S, Hanif HM (2020) Pancytopenia Resulting from Renal Osteodystrophy as A Consequence of Secondary Hyperparathyroidism. JSM Clin Case Rep 8(2): 1180. Meraj F, et al. (2020) Central Table 1: for reporting bone biopsy specimens [1]. The following three of Types of Renal Osteodystrophy.Turnover Mineralization Volume Mild hyperparathyroid- parametersbone parameters in the are four used: types turnover, of renal osteodystrophymineralization and (Table volume. 1). Moderate Normal Normal The following table illustrates the differences in these three related bone disease Osteitis fibrosa cystica Normal Osteitis fibrosa cystica (which represents our current clinical Adynamic bone disease High Normal High normal case) is histologically characterized by bone marrow fibrosis, Low to In addition, there is increased bone resorption, manifested by Low Osteomalacia Abnormal with extension of fibrous tissue and osteoclasts into trabeculae. medium Low to Mixed uremic Low Abnormal Normal cellssurface are excavation also seen in (Howship’s increased numbers lacunae [6].containing osteoclasts). osteodystrophy Hemosiderin laden macrophages and foreign body-type giant High CLINICAL PRESENTATION We describe the case of a 24 year old female with End Stage Renal Disease, on haemodialysis since the age of 21 years. She presented with complains of swelling around the site of nutritionalher arteriovenous supplements fistula (vitamin on her B12,left armfolic sinceacid and2-3 calcium),months. She was on weekly erythropoietin, antihypertensives and red cell concentrates since 2 years. CBC showed normocytic, despite which she was receiving with fortnightly to monthly normochromic anemia (Haemoglobin 5.4g/dl, WBC count level3.98x10^9/L was within and normal Platelet limits. count Her114x10^9/L). serum parathyroid Reticulocyte hormone count was 2.4%, serum B12 was raised (>2000pg/ml) and RBC folate level was markedly elevated (1140.90pg/ml; reference range: 15-68pg/ml). Pre-dialysis biochemical tests included raised serum creatinine(9.06mg/dl), normal serum calcium(9.1mg/dl), hyperphosphatemia (5.4mg/dl) and raised alkaline phosphatase (543 U/L). Serum vitamin D was severely deficient (7.2ng/ml). Ultrasound Abdomen showed no visceromegaly, reduced hepatic parenchymal echogenecity, pelvic ascites and bilateral atrophic kidneys. Computed tomography of the chest showed bilateral atelectatic changes in the lungs. Figure 1 She was transfused several units of red cell concentrates; Low power view (x4) showing irregular bony trabeculae and However, with time she progressed into pancytopenia replacement of hematopoietic marrow by fibrous tissue. performed(Haemoglobin to evaluate 9.7g/dl, theWBC cause count of 2.1x10^9/L pancytopenia. and Platelet count rtarate-r 48x10^9/L). Bone marrow aspiration and trephine biopsy were RESULTS staining for TRAP (ta esistant acid phosphatase) [Figure 3], andIn theincreased involved vascularity cores, reticulin was highlighted stain showed by CD34. diffuse and The bone marrow aspirate was a dry tap with scattered neutrophils, lymphocytes and few erythroid precursors. However, touch imprints of trephine biopsy showed a cellular specimen dense increase in reticulin with extensive intersections (Grade MF-02) (Figure 4). Overall clinical and bone marrow findings, along with exhibiting trilineage haematopoiesis, with normoblastic secondary to hyperparathyroidism. hyperparathyroidism, are suggestive of renal osteodystrophy erythropoiesis, all stages of myeloid maturation and adequate DISCUSSION megakaryocytes.Haemotoxylin Few and scattered Eosin osteoclastsstained sections were also of noted. trephine hyperparathyroidism, associated with pancytopenia and biopsy showed three cores, comprising of soft tissue, cortex suboptimalOur case response illustrates to erythropointinan example of and marked hematinics, secondary due and good length subcortical and medullary marrow. One core showed cellular marrow with trilineage haematopoiesis. The patients, parathyroidectomy has been shown to improve anemia other cores were markedly hypocellular and showed irregular to replacement of cellular marrow by fibrous tissue. In these bony trabeculae with prominent fibrous tissue and osteoclasts (Figure 1). Prominent resorption bays (“Howship’s lacunae”), and reduce the requirements for exogenous erythropoietin therapyTwo [7].similar cases have been reported in recent literature. tunneling into trabeculae by fibrous tissue, haemosiderin laden Yeo JH, Islam A reported the case of a 24-year-old female who macrophages and increased vascularity were also noted [Figure 2]. Osteoclasts were highlighted by immunohistochemical JSM Clin Case Rep 8(2): 1180 (2020) 2/4 Meraj F, et al. (2020) Central A B C Figure 2 Higher power view (x10) showing tunneling into trabeculae by fibrous tissue (Figure A, B; red arrows), irregular bony trabeculae (Figure C) and surface excavation of bony trabeculae (Howships’s lacunae) (Figure C: red arrows). Figure 4 Figure 3 Immunohistochemistry for TRAP (tartarate-resistant acid Reticulin stain showing myelofibrosis. phosphatase) highlighting positivity in scattered osteoclasts. binders [10]. In addition, vitamin D deficiency is treated. despite erythropoietin injections and adequate supplementation Specific treatment options for hyperparathyroidism include developed worsening pancytopenia while on haemodialysis, calcimimetics, calcitriol, synthetic vitamin D analogs or a combination of these agents. The goal is to maintain
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