Idiopathic, immune, infectious, and idiosyncratic neutropenias

Palmblad, Jan; Borne, A. E. G. K. Von Dem

doi:10.1053/shem.2002.31919

Cited by 54 publications

(27 citation statements)

References 76 publications

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“…Performance of bone marrow aspiration and trephine biopsy in deep sedation was strongly encouraged. Indirect anti-neutrophil antibodies detection by flow cytometry was agreed on to be the most useful and practical method for identifying the autoimmune neutropenia (AIN) [1,7,9,17,[42][43][44][45][46]. The low sensitivity (74% at first assessment) [45] of this method was recognized by the panel but it was considered, in the respect of diagnostic power, less detrimental than the lower specificity (very high number of false positives) of the direct method [44] (III, V, EO, 9, A).…”

Section: (Eo 9 A)mentioning

confidence: 99%

“…At this stage of the pathway, if no maturation block on marrow morphology is detected, and the indirect antibodies test against neutrophils are negative at least four times, the diagnosis of idiopathic neutropenia (IN) is considered appropriate [1,[7][8][9]17,46] (EO, 9,A). Since IN still remains an exclusion diagnosis the panel agreed on the need to place IN patients on a monitoring program and to perform diagnostic re-evaluation in case new elements suggestive of other diagnosis arise (EO, 9, A).…”

Section: (Eo 9 A)mentioning

confidence: 99%

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Congenital and acquired neutropenia consensus guidelines on diagnosis from the Neutropenia Committee of the Marrow Failure Syndrome Group of the AIEOP (Associazione Italiana Emato‐Oncologia Pediatrica)

Fioredda

Calvillo

Bonanomi

et al. 2011

Pediatric Blood & Cancer

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Congenital and acquired neutropenia are rare disorders whose frequency in pediatric age may be underestimated due to remarkable differences in definition or misdiagnosed because of the lack of common practice guidelines. Neutropenia Committee of the Marrow Failure Syndrome Group (MFSG) of the AIEOP (Associazione Italiana Emato‐Oncologia Pediatrica) elaborated this document following design and methodology formerly approved by the AIEOP board. The panel of experts reviewed the literature on the topic and participated in a conference producing a document which includes a classification of neutropenia and a comprehensive guideline on diagnosis of neutropenia. Pediatr Blood Cancer 2011;57:10–17. © 2011 Wiley‐Liss, Inc.

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Section: (Eo 9 A)mentioning

confidence: 99%

Section: (Eo 9 A)mentioning

confidence: 99%

Congenital and acquired neutropenia consensus guidelines on diagnosis from the Neutropenia Committee of the Marrow Failure Syndrome Group of the AIEOP (Associazione Italiana Emato‐Oncologia Pediatrica)

Fioredda

Calvillo

Bonanomi

et al. 2011

Pediatric Blood & Cancer

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“…Alemtuzumab is a humanized IgG1k monoclonal antibody that recognizes the CD52 antigen on human lymphocytes, monocytes, macrophages, eosinophils, dendritic cells and natural killer cells. Alemtuzumab causes cell lysis via complement or antibody-dependent cellular cytotoxicity as well as by directly acting on the T lymphocytes, which play an important role in controlling the expansion of antibody producing autoreactive B-cell clones (15). In the case of our patient, who had refractory P-AIN, Alemtuzumab was effective in maintaining stable neutrophil counts and reducing neutropenia-related hospitalizations.…”

Section: Discussionmentioning

confidence: 69%

“…According to Palmblad et al, a number of patients with severe P-AIN have demonstrated transient responses to Filgrastim, corticosteroids, or antilymphocyte globulin, whereas treatment with Cyclosporine, Azathioprine and IVIG has been shown to be unsuccessful (15). Alemtuzumab is the only therapeutic agent that has been shown to induce a sustained response (2,15). Alemtuzumab is a humanized IgG1k monoclonal antibody that recognizes the CD52 antigen on human lymphocytes, monocytes, macrophages, eosinophils, dendritic cells and natural killer cells.…”

Section: Discussionmentioning

confidence: 99%

Refractory Adult Primary Autoimmune Neutropenia that Responded to Alemtuzumab

et al. 2016

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Primary autoimmune neutropenia (P-AIN) is an extremely rare disease. The most effective treatment for primary P-AIN is a granulocyte colony-stimulating factor; however, no curative treatment has been reported. We herein report a case of an adult P-AIN patient with a relatively mild medical history (irrespective of the severe neutropenia) who showed a sustained hematological response over seventeen months after the initiation of treatment with subcutaneous Alemtuzumab.

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“…The inability to detect an immune-mediated process or other obvious cause often leads to diagnosis of chronic idiopathic neutropenia (CIN) [1][2][3][4][5][6]. Similarly, secondary autoimmune neutropenia (AIN), usually associated with collagen vascular diseases, lymphoproliferative disorders and viruses, is typically diagnosed by exclusion and thus relies on the quality of clinical and laboratory workups [6][7][8][9].…”

Section: Materials and Methods-using Rational Algorithms For T-cell Rmentioning

confidence: 99%

Clonal predominance of CD8+ T cells in patients with unexplained neutropenia

Wlodarski

Nearman

Jiang

et al. 2008

Experimental Hematology

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Objective-T-cell-mediated autoimmunity may be involved in some cases of idiopathic neutropenia. We hypothesized that a precise T-cell receptor repertoire analysis may uncover cytotoxic T-cell (CTL) expansions that are less pronounced than those seen in T large granular lymphocyte leukemia (T-LGL), but are pathophysiologically analogous and thus can serve as markers of a T-cell-mediated process. Materials and Methods-Using rational algorithms for T-cell receptor analysis and in vivotracking of CTL responses previously established in our laboratory, we studied patients with unexplained chronic neutropenia (n = 20), T-LGL (n = 15), and healthy controls (n = 12). We further investigated the involvement of soluble inhibitory factors by coculture assays. To determine the level of immune activation, we studied interferon-g expression in CD8 + cells using Taqman polymerase chain reaction.Results-Fifteen expanded (immunodominant) CTL clones were detected in 12 of 20 patients. In comparison to LGL leukemia, these clones were less immunodominant, but clearly discernible from subclinical lymphoproliferations in controls. As a surrogate of cytotoxic activity, we found markedly increased production of interferon-γ in most of the neutropenia patients, irrespective of the presence of immunodominant CTL clones.Conclusions-These results suggest that, while immunodominant CTL clones are detectable in a proportion of patients only, CTL-mediated pathophysiology may be a general mechanism operating in idiopathic neutropenia. Oligogoclonal CTL expansions in chronic neutropenia may indicate an ongoing autoimmune process, while highly polarized monoclonalities in a subset of neutropenic LGL patients may represent the "extreme" end of the clonal continuum.Drugs, hereditary factors, infections, and intrinsic bone marrow diseases explain the etiology of most neutropenias. The inability to detect an immune-mediated process or other obvious cause often leads to diagnosis of chronic idiopathic neutropenia (CIN) [1][2][3][4][5][6]. Similarly, secondary autoimmune neutropenia (AIN), usually associated with collagen vascular diseases, lymphoproliferative disorders and viruses, is typically diagnosed by exclusion and thus relies on the quality of clinical and laboratory workups [6][7][8][9]. Lineage-restricted cytopenias, including neutropenia, have been associated with T-cell large granular lymphocyte leukemia It is likely that patients with AIN/CIN may belong to a heterogeneous group of diseases combining various autoimmune etiologies. Appearance of the bone marrow may reveal some clues as to the cellular targets of the autoimmune process; left shift could indicate that more mature cells are targets, while pure white cell aplasia suggests that very early myeloid precursors are affected. The similar target spectrum in AIN/CIN and T-LGL suggests that cytotoxic T-cell (CTL)-mediated autoimmunity can also operate in AIN/CIN. Consequently, detection of highly polarized CTL expansions in some cases of neutropenia patients may be consistent wi...

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Idiopathic, immune, infectious, and idiosyncratic neutropenias

Cited by 54 publications

References 76 publications

Congenital and acquired neutropenia consensus guidelines on diagnosis from the Neutropenia Committee of the Marrow Failure Syndrome Group of the AIEOP (Associazione Italiana Emato‐Oncologia Pediatrica)

Congenital and acquired neutropenia consensus guidelines on diagnosis from the Neutropenia Committee of the Marrow Failure Syndrome Group of the AIEOP (Associazione Italiana Emato‐Oncologia Pediatrica)

Refractory Adult Primary Autoimmune Neutropenia that Responded to Alemtuzumab

Clonal predominance of CD8+ T cells in patients with unexplained neutropenia

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