PMC5491819_01
Bone marrow findings on admission. (A) The clot section of the bone marrow aspirate shows mildly hypercellular bone marrow with an increased number of magakaryocytes and erythroblasts (Hematoxylin and Eosin staining, x100). (B) Immunostaining with anti-CD71-antibody confirms the increase of erythroid cells (x200). (C) Iron stain demonstrates an increased number of sideroblasts (x200). (D) The bone marrow smear shows increased immature erythroid cells with occasional binucleated erythroblasts (Wright-Giemsa stain x400). (E) The arrow indicates a micromegakaryocyte (Wright-Giemsa stain x400). (F) Iron stain shows increased sideroblasts, of which 45% were ring sideroblasts (arrows) (x1,000).
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The BM was mildly hypercellular and the magakaryocytes had increased in number and showed a variety of dysplastic features, such as micromegakaryocytes and multiple widely separated nuclei (Fig. 1A and E). The erythroid cells accounted for 60% of the BM nucleated cells and the number of immature cells had increased with occasional binucleated erythroblasts (Fig. 1A, B, and D). Iron staining showed most of the erythroid cells to be sideroblasts, of which 45% were ring sideroblasts (Fig. 1C and F).
Computed tomography (CT) and magnetic resonance imaging (MRI) of the presacral extramedullary hematopoiesis. (A) Contrast-enhanced CT reveals a lobulated and well-margined mass (arrows) in the presacral region. (B-D) MRI shows that the mass (arrows) is iso- or high-intensity on T1-weighted (B) and hypo-intensity on T2-weighted (C) images and that it is diffusely enhanced after the intravenous injection of gadolinium (D).
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Computed tomography (CT) was performed to evaluate the hepatosplenomegaly detected on a physical examination and revealed a lobulated and well-margined mass measuring 3x5 cm in size in the presacral region which was diffusely contrast-enhanced (Fig. 2A). Magnetic resonance imaging (MRI) showed the presacral mass to have iso- or high-intensity on T1-weighted and hypo-intensity on T2-weighted images and it was diffusely enhanced after the intravenous injection of gadolinium (Fig. 2B-D).
Computed tomography-guided biopsy of the presacral extramedullary hematopoiesis. (A) A biopsy sample reveals hematopoietic tissue with hyperplasia of the erythroid cells (Hematoxylin and Eosin staining, x100). (B) Immunostaining shows the number of CD71-positive erythroid cells to have increased (x200). (C) The number of CD42b-positive magakaryocytes also increased and the presence of small mononuclear megakaryocytes and those with widely separated nuclei are observed (x200). (D) An increase in the number of p53-postive cells (x200) is also observed.
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The biopsy revealed the presence of hematopoietic tissue with a fat:cell ratio of 1:1-2 (Fig. 3A). CD71-positive erythroid cells were hyperplastic (Fig. 3B) and the number of CD42b-positive magakaryocytes were also found to have increased and the presence of small mononuclear megakaryocytes was also noted (Fig. 3C). There was no increase in the number of myeloblasts; however, the number of p53-positive cells had increased (Fig. 3D).