Massive hyper-reactive hematopoietic nests in bilateral iliac bones in a patient with mild aplastic anemia

Nishimura, Ryosei; Mase, Shintaro; Araki, Raita; Fujiki, Toshihiro; Kuroda, Rie; Maeba, Hideaki; Koizumi, Satoshi; Yachie, Akihiro

doi:10.1002/pbc.25140

Cited by 6 publications

(4 citation statements)

References 6 publications

(6 reference statements)

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“…Although the detection of a hypocellular BM is a prerequisite for diagnosing AA, assessing BM cellularity in patients with BM failure can be difficult, particularly when the cytopenia is not severe. Even when the BM of a given bone site is grossly replaced with fat tissue as a result of the immune-mediated destruction of HSPCs, some hematopoietic nests may remain in other bone sites and may show hypercellularity due to increased BM activity that compensates for the decreased hematopoiesis [7]. BM aspiration or biopsies of these hot spots may produce erroneous results.…”

Section: Introductionmentioning

confidence: 98%

Diagnostic problems in acquired bone marrow failure syndromes

Nakao

2016

Int J Hematol

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The dysplastic signs in PNH patients can be ascribed to co-existing MDS clones, which over time can give rise to acute myeloid leukemia. Abnormal HSPC clones may be present and exhibit dysplastic signs at the diagnosis of BM failure-type PNH or subclinical PNH (PNHsc). However, PNH-phenotype cells are rarely detected in patients in bona fide preleukemic states, such as RARS and RAEB [2]. A recent genomic analysis of hemolytic PNH patients revealed a low incidence of genomic abnormalities associated with MDS [3]. A genomic analysis of a large number of AA patients recently published by our group revealed a lower frequency of abnormalities in MDS-related genes, such as TP53 and RUNX1, in AA patients with PIGA mutations than in patients without PIGA mutations [4]. It is thus unlikely that the dysplastic signs in PNH patients represent co-existing abnormal MDS clones.Dysplastic signs in immature blood cells can be caused by extrinsic factors, such as nutritional deficiencies and toxins. Some patients with collagen diseases show dysplastic signs of immature cells similar to those of patients with MDS. In patients with AA characterized by small populations of PNH-type cells, dysplastic features typically disappear after they achieve hematologic remission following immunosuppressive therapy [5]. Reversible dysplasia, such as occurs in inflammatory diseases and immune-mediated BM failure, suggests that inflammatory cytokines produced locally in the BM may induce dysplastic changes in immature blood cells. It is thus important to bear in mind that the immune pathophysiology of BM failure, which allows PIGA-mutant HSPCs to contribute to hematopoiesis, is often associated with some degree of erythroid and granulocytic dysplasia.

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Section: Introductionmentioning

confidence: 98%

Diagnostic problems in acquired bone marrow failure syndromes

Nakao

2016

Int J Hematol

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“…Certain aspects of these criteria warranted revision: for example, platelet-associated IgG (PAIgG), as used here, was of little diagnostic value due to a lack of specificity [ 1 ]; while bone marrow examination, which is considered necessary, does not yield findings specific to ITP [ 2 ]. Most notably, aplastic anemia can be misdiagnosed as ITP when anemia is preceded by thrombocytopenia in the early stage, and bone marrow examination does not always reveal hypoplasia depending on the puncture site [ 3 ]. Roughly 10% of cases diagnosed as refractory ITP are bone marrow failure [ 4 ], and differentiating ITP from aplastic anemia is considered more important in Japan, where the prevalence of aplastic anemia is markedly higher that in the West [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Reference guide for the diagnosis of adult primary immune thrombocytopenia, 2023 edition

Kashiwagi,

Kuwana,

Murata

et al. 2023

Int J Hematol

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Primary immune thrombocytopenia (ITP) is an autoimmune disorder characterized by isolated thrombocytopenia due to accelerated platelet destruction and impaired platelet production. Diagnosis of ITP is still challenging because ITP has been diagnosed by exclusion. Exclusion of thrombocytopenia due to bone marrow failure is especially important in Japan because of high prevalence of aplastic anemia compared to Western countries. Hence, we propose a new diagnostic criteria involving the measurement of plasma thrombopoietin (TPO) levels and percentage of immature platelet fraction (RP% or IPF%); 1) isolated thrombocytopenia with no morphological evidence of dysplasia in any blood cell type in a blood smear, 2) normal or slightly increased plasma TPO level (< cutoff), 3) elevated RP% or IPF% (> upper limit of normal), and 4) absence of other conditions that potentially cause thrombocytopenia including secondary ITP. A diagnosis of ITP is made if conditions 1-4 are all met. Cases in which criterion 2 or 3 is not met or unavailable are defined as “possible ITP,” and diagnosis of ITP can be made mainly by typical clinical course. These new criteria enable us to clearly differentiate ITP from aplastic anemia and other forms of hypoplastic thrombocytopenia and can be highly useful in clinical practice for avoiding unnecessary bone marrow examination as well as for appropriate selection of treatments.

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“…1 Acquired bone marrow failure is termed idiopathic when there is no identified exposure capable of damaging the bone marrow and when there is no relevant nutritional deficiency such as vitamin B 12 or folate (Identification differs from causality. Not all exposures may be known to the subject and/or the haematologist).…”

Section: Are Mild/moderate Acquired Idiopathic Aplastic Anaemia And/omentioning

confidence: 99%

Are mild/moderate acquired idiopathic aplastic anaemia and low-risk myelodysplastic syndrome one or two diseases or both and how should it/they be treated?

Nakao

Gale

2016

Leukemia

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Massive hyper-reactive hematopoietic nests in bilateral iliac bones in a patient with mild aplastic anemia

Cited by 6 publications

References 6 publications

Diagnostic problems in acquired bone marrow failure syndromes

Diagnostic problems in acquired bone marrow failure syndromes

Reference guide for the diagnosis of adult primary immune thrombocytopenia, 2023 edition

Are mild/moderate acquired idiopathic aplastic anaemia and low-risk myelodysplastic syndrome one or two diseases or both and how should it/they be treated?

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