Reversible Myelofibrosis in Pediatric Renal Osteodystrophy

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A 9-year-old female with no medical care for 3 years presented with recurrent emesis,
epistaxis, and pallor. Laboratory evaluation showed renal failure with blood urea
nitrogen 216 mg/dL and serum creatinine of 17.5 mg/dL. Hematologic workup showed severe
normocytic anemia with hemoglobin concentration of 4.3 g/dL and an initial platelet
count of 108 x 10
9/L that decreased to 60 x 10
9/L over 5 days. Her total white blood cell count was normal, but she had mild lymphopenia
(1.3 X 10
9/L). Parathyroid hormone (PTH) levels were markedly elevated at 1600 pg/mL (normal
range 15-87). Peripheral blood smear review showed normocytic normochromic anemia
with moderate anisopoikilocytosis, including rare acanthocytes, tear drop cells, and
rare schistocytes. No blasts were present. The cause of renal failure was not determined
but the patient had small bilateral kidneys on ultrasound, consistent with long-standing
chronic kidney disease (CKD), possibly secondary to renal dysplasia. Bone marrow biopsy
revealed hypocellularity (30%) and grade 2 nonuniform, patchy peritrabecular myelofibrosis
with increased osteoclastic and osteoblastic activity (
Figure, A). No immunophenotypic or cytogenetic abnormalities were detected and testing for

JAK2V617F was negative. The bone marrow findings in the setting of hyperparathyroidism due
to long-standing CKD and absence of other etiologies were consistent with non-malignant
myelofibrosis due to secondary hyperparathyroidism/renal osteodystrophy. The patient
promptly underwent hemodialysis and was treated with paricalcitol for hyperparathyroidism.
Platelet levels improved within two weeks and hemoglobin normalized within three months
of initiating erythropoiesis-stimulating agent (Figure, C). PTH levels decreased within
2 months and remained

Figure thumbnail gr1

Figure 1
A) Bone marrow biopsy image showing hypocellular marrow with peritrabecular myelofibrosis,
increased osteoclastic and osteoblastic activity, and anastomosing trabeculae at time
of presentation. B) Normocellular marrow with no fibrosis and normal trabeculae after
initiation of hemodialysis and control of hyperparathyroidism. C) Changes in hemoglobin
concentration, platelet count, and parathyroid hormone (PTH) levels over time.

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