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2.
Isr Med Assoc J ; 23(8): 501-505, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34392626

RESUMO

BACKGROUND: Multiple myeloma (MM) affects the long bones in 25% of patients. The advent of positron-emission tomography/computed tomography (PET/CT) scanners offers the possibility of both metabolic and radiographic information and may help determine fracture risk. To the best of our knowledge, no published study correlates these two factors with long bone fractures. OBJECTIVES: To evaluate the impact of PET/CT on fracture risk assessment in multiple myeloma patients. METHODS: We identified all bone marrow biopsy proven multiple myeloma patients from 1 January 2010 to 31 January 2015 at a single institution. We prospectively followed patients with long bone lesions using PET/CT scan images. RESULTS: We identified 119 patients (59 males/60 females) with 256 long bone lesions. Mean age at diagnosis was 58 years. The majority of lesions were in the femur (n=150, 59%) and humerus (n=84, 33%); 13 lesions in 10 patients (8%) required surgery for impending (n=4) or actual fracture (n=9). Higher median SUVmax was measured for those with cortical involvement (8.05, range 0-50.8) vs. no involvement (5.0, range 2.1-18.1). SUVmax was found to be a predictor of cortical involvement (odds ratio = 1.17, P = 0.026). No significant correlation was found between SUVmax and pain or fracture (P = 0.43). CONCLUSIONS: Improved medical treatment resulted improvement in 8% of patients with an actual or impending fracture. The orthopedic surgeons commonly use the Mirels classification for long bone fracture prediction. Adding PET/CT imaging to study in myeloma long bone lesions did not predict fracture risk directly but suggested it indirectly by cortical erosion.


Assuntos
Fraturas do Fêmur , Fraturas do Úmero , Mieloma Múltiplo , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Medição de Risco/métodos , Biópsia/métodos , Medula Óssea/patologia , Osso Cortical/diagnóstico por imagem , Osso Cortical/patologia , Feminino , Fraturas do Fêmur/diagnóstico , Fraturas do Fêmur/etiologia , Fraturas do Fêmur/cirurgia , Fluordesoxiglucose F18/farmacologia , Fixação de Fratura/métodos , Fixação de Fratura/estatística & dados numéricos , Humanos , Fraturas do Úmero/diagnóstico , Fraturas do Úmero/etiologia , Fraturas do Úmero/cirurgia , Israel/epidemiologia , Masculino , Pessoa de Meia-Idade , Mieloma Múltiplo/complicações , Mieloma Múltiplo/diagnóstico por imagem , Mieloma Múltiplo/patologia , Valor Preditivo dos Testes , Compostos Radiofarmacêuticos/farmacologia , Estudos Retrospectivos
3.
Cell Death Dis ; 12(8): 762, 2021 08 03.
Artigo em Inglês | MEDLINE | ID: mdl-34344867

RESUMO

While vaccination is the single most effective intervention to drastically reduce severe disease and death following SARS-CoV-2 infection, as shown in UK and Israel, some serious concerns have been raised for an unusual adverse drug reaction (ADR), including vaccine-induced immune thrombotic thrombocytopenia (VITT) with concurrent low platelets as well as capillary leak syndrome. In fact, the overall safety of the vaccine is highlighted by the low frequency of ADR considering that in UK, by the early June, 40 million first doses and 29 million second doses have been injected; nonetheless, 390 thrombotic events, including 71 fatal events have been reported. Interestingly, the cases reported low platelet counts with the presence of anti-platelet factor-4 (PF4) antibodies, indicating an abnormal clotting reaction. Here, out of three referred cases, we report a post-vaccine clinical case of fatal thrombosis with postmortem examination and whole exome sequencing (WES) analysis, whose pathogenesis appeared associated to a preexisting condition of thrombocytopenia due to myelodysplasia.


Assuntos
Vacinas contra COVID-19/efeitos adversos , Trombocitopenia/complicações , Tromboembolia/etiologia , Medula Óssea/patologia , COVID-19/prevenção & controle , Evolução Fatal , Feminino , Humanos , Pulmão/patologia , Pessoa de Meia-Idade , Síndromes Mielodisplásicas/complicações , Síndromes Mielodisplásicas/diagnóstico , Síndromes Mielodisplásicas/patologia , SARS-CoV-2/imunologia , Trombocitopenia/diagnóstico , Trombocitopenia/patologia
4.
Hematology ; 26(1): 594-600, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34402416

RESUMO

BACKGROUND: Among myeloproliferative neoplasms, it is often difficult to distinguish essential thrombocythaemia (ET) from prefibrotic-stage primary myelofibrosis (PMF) with thrombocytosis given their overlapping clinicopathological phenotypes. CASE PRESENTATION: We encountered a 45-year-old male who was initially diagnosed with ET and eventually became transformed to secondary myelofibrosis 20 years later. Two distinct types of aberrant megakaryocytes were observed at diagnosis: one type characteristic of ET and the other type characteristic of PMF. With a proliferation in the bone marrow, aberrant megakaryocytes were infiltrated into the extramedullary organs and were even present in the thrombus were observed at autopsy. As a result of next-generation sequencing, the significant increase of variant allele frequency (VAF) of JAK2 V617F and U2AF1 S34Y mutations was observed in the bone marrow cells at the final stage. CONCLUSIONS: This patient could be recognized as an atypical case of aggressive megakaryocytosis transformed from ET.


Assuntos
Megacariócitos/patologia , Mielofibrose Primária/patologia , Trombocitemia Essencial/patologia , Medula Óssea/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Mielofibrose Primária/complicações , Mielofibrose Primária/diagnóstico , Trombocitemia Essencial/complicações , Trombocitemia Essencial/diagnóstico
5.
Immunity ; 54(7): 1511-1526.e8, 2021 07 13.
Artigo em Inglês | MEDLINE | ID: mdl-34260887

RESUMO

Myeloid cells encounter stromal cells and their matrix determinants on a continual basis during their residence in any given organ. Here, we examined the impact of the collagen receptor LAIR1 on myeloid cell homeostasis and function. LAIR1 was highly expressed in the myeloid lineage and enriched in non-classical monocytes. Proteomic definition of the LAIR1 interactome identified stromal factor Colec12 as a high-affinity LAIR1 ligand. Proteomic profiling of LAIR1 signaling triggered by Collagen1 and Colec12 highlighted pathways associated with survival, proliferation, and differentiation. Lair1-/- mice had reduced frequencies of Ly6C- monocytes, which were associated with altered proliferation and apoptosis of non-classical monocytes from bone marrow and altered heterogeneity of interstitial macrophages in lung. Myeloid-specific LAIR1 deficiency promoted metastatic growth in a melanoma model and LAIR1 expression associated with improved clinical outcomes in human metastatic melanoma. Thus, monocytes and macrophages rely on LAIR1 sensing of stromal determinants for fitness and function, with relevance in homeostasis and disease.


Assuntos
Homeostase/fisiologia , Pulmão/metabolismo , Macrófagos Alveolares/metabolismo , Monócitos/metabolismo , Receptores Imunológicos/metabolismo , Animais , Apoptose/fisiologia , Medula Óssea/metabolismo , Medula Óssea/patologia , Células COS , Diferenciação Celular/fisiologia , Linhagem Celular , Linhagem Celular Tumoral , Linhagem da Célula/fisiologia , Proliferação de Células/fisiologia , Chlorocebus aethiops , Feminino , Humanos , Pulmão/patologia , Macrófagos Alveolares/patologia , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Monócitos/patologia , Células Mieloides/metabolismo , Células Mieloides/patologia , Metástase Neoplásica/patologia , Proteômica/métodos , Transdução de Sinais/fisiologia
6.
Int J Lab Hematol ; 43 Suppl 1: 54-64, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34288436

RESUMO

Ever since hematopoietic cells became "events" enumerated and characterized in suspension by cell counters or flow cytometers, researchers and engineers have strived to refine the acquisition and display of the electronic signals generated. A large array of solutions was then developed to identify at best the numerous cell subsets that can be delineated, notably among hematopoietic cells. As instruments became more and more stable and robust, the focus moved to analytic software. Almost concomitantly, the capacity increased to use large panels (both with mass and classical cytometry) and to apply artificial intelligence/machine learning for their analysis. The combination of these concepts raised new analytical possibilities, opening an unprecedented field of subtle exploration for many conditions, including hematopoiesis and hematological disorders. In this review, the general concepts and progress achieved in the development of new analytical approaches for exploring high-dimensional data sets at the single-cell level will be described as they appeared over the past few years. A larger and more practical part will detail the various steps that need to be mastered, both in data acquisition and in the preanalytical check of data files. Finally, a step-by-step explanation of the solution in development to combine the Bioconductor clustering algorithm FlowSOM and the popular and widely used software Kaluza® (Beckman Coulter) will be presented. The aim of this review was to point out that the day when these progresses will reach routine hematology laboratories does not seem so far away.


Assuntos
Citometria de Fluxo/métodos , Neoplasias Hematológicas/diagnóstico , Aprendizado de Máquina não Supervisionado , Algoritmos , Medula Óssea/metabolismo , Medula Óssea/patologia , Análise por Conglomerados , Biologia Computacional/métodos , Progressão da Doença , Neoplasias Hematológicas/etiologia , Humanos , Modelos Teóricos , Software
7.
Int J Lab Hematol ; 43 Suppl 1: 23-28, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34288439

RESUMO

This paper aims to emphasize the importance of applying international consensus guidelines to detect qualitative and quantitative abnormalities of megakaryocytes on smears of bone marrow aspirates (BMA) for a shared and harmonized diagnostic path between different laboratories. Careful evaluation of megakaryocytes on BMA smears represents a cornerstone in the diagnosis of most clonal and nonclonal hematological diseases. Images associated with the detailed morphologic description of normal, reactive, abnormal, and dysplastic megakaryocytes are also reported together with examples of similar cells that, if not promptly identified, can lead to a morphological misdiagnosis.


Assuntos
Células da Medula Óssea/citologia , Células da Medula Óssea/patologia , Medula Óssea/patologia , Megacariócitos/citologia , Megacariócitos/patologia , Biópsia por Agulha , Diagnóstico Diferencial , Histocitoquímica/métodos , Humanos , Microscopia/métodos , Trombopoese
8.
Int J Lab Hematol ; 43 Suppl 1: 78-81, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34288445

RESUMO

Philadelphia (BCR-ABL)-negative myeloproliferative neoplasms (MPNs) include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). MPN can transform into an accelerated or a blast phase, which is associated with poor response to standard therapy and low overall median survival. We present an interesting case of a patient with a history of PMF and progression and summarize the current studies on genetic features of myeloproliferative neoplasms in blast phase (MPN-BP) with an emphasis on PMF. Although MPN-BP show ≥20% blasts in peripheral blood or bone marrow, it is not considered as acute myeloid leukemia (AML) according to the WHO classification. While MPNs-BP typically lack genetic mutations seen in de novo AML, they commonly harbor IDH1/2, SRSF2, ASXL1, and TP53 mutations, similar to the genetic profiles of acute myeloid leukemia with myelodysplasia-related changes (AML-MRC).


Assuntos
Suscetibilidade a Doenças , Mielofibrose Primária/diagnóstico , Mielofibrose Primária/etiologia , Biomarcadores , Medula Óssea/patologia , Análise Citogenética , Progressão da Doença , Índices de Eritrócitos , Predisposição Genética para Doença , Humanos , Imunofenotipagem , Pessoa de Meia-Idade , Mielofibrose Primária/metabolismo , Mielofibrose Primária/terapia
9.
Int J Lab Hematol ; 43 Suppl 1: 82-85, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34288451

RESUMO

Unexplained blood cytopenias, in particular anemia, are often found in older individuals. The relationship between these cytopenias and myeloid neoplasms like myelodysplastic syndromes is currently poorly defined. Terminology used to describe patients with unexplained cytopenias and with clonally restricted hematopoiesis can be confusing and is evolving. This review uses a complex clinical case with borderline morphology and somatic mutations with high variant allele frequencies to illustrate a diagnostic approach to clonal cytopenias, and differentiation from myeloid neoplasms with a focus on appropriate ancillary testing. Testing for somatic mutations and variant allele frequency is helpful in assessing risk for progression to myeloid malignancy. The interpretation of mutation profiles in patients with cytopenia has been challenging, as some of these genes are commonly detected in elderly adults showing a normal blood count as well as in individuals with nonmalignant bone marrow failure syndromes. For patients with unexplained cytopenias, longitudinal follow-up including monitoring of blood counts may also be appropriate.


Assuntos
Hematopoiese Clonal , Pancitopenia/diagnóstico , Pancitopenia/etiologia , Idoso , Biomarcadores , Medula Óssea/patologia , Hematopoiese Clonal/genética , Diagnóstico Diferencial , Gerenciamento Clínico , Suscetibilidade a Doenças , Testes Hematológicos , Humanos , Imunofenotipagem , Masculino
10.
Curr Opin Hematol ; 28(5): 364-371, 2021 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-34232140

RESUMO

PURPOSE OF REVIEW: Bone marrow fibrosis is the progressive replacement of blood-forming cells by reticulin fibres, caused by the acquisition of somatic mutations in hematopoietic stem cells. The molecular and cellular mechanisms that drive the progression of bone marrow fibrosis remain unknown, yet chronic inflammation appears to be a conserved feature in most patients suffering from myeloproliferative neoplasms. RECENT FINDINGS: Here, we review recent literature pertaining to the role of inflammation in driving bone marrow fibrosis, and its effect on the various hematopoietic and nonhematopoietic cell populations. SUMMARY: Recent evidence suggests that the pathogenesis of MPN is primarily driven by the hematopoietic stem and progenitor cells, together with their mutated progeny, which in turn results in chronic inflammation that disrupts the bone marrow niche and perpetuates a disease-permissive environment. Emerging data suggests that specifically targeting stromal inflammation in combination with JAK inhibition may be the way forward to better treat MPNs, and bone marrow fibrosis specifically.


Assuntos
Medula Óssea , Neoplasias Hematológicas , Células-Tronco Hematopoéticas , Mielofibrose Primária , Medula Óssea/metabolismo , Medula Óssea/patologia , Neoplasias Hematológicas/tratamento farmacológico , Neoplasias Hematológicas/metabolismo , Neoplasias Hematológicas/patologia , Células-Tronco Hematopoéticas/metabolismo , Células-Tronco Hematopoéticas/patologia , Humanos , Inflamação/tratamento farmacológico , Inflamação/metabolismo , Inflamação/patologia , Transtornos Mieloproliferativos/tratamento farmacológico , Transtornos Mieloproliferativos/metabolismo , Transtornos Mieloproliferativos/patologia , Mielofibrose Primária/tratamento farmacológico , Mielofibrose Primária/metabolismo , Mielofibrose Primária/patologia
11.
J Radiol Case Rep ; 15(6): 19-25, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-34276877

RESUMO

A 66-year-old cachectic female with underlying anorexia nervosa and lower limb weakness was referred for a spinal Magnetic Resonance Imaging. Imaging appearances were initially thought to represent underlying systemic pathology involving bone marrow or inadvertent wrong selection of imaging sequences. It was, however, established that unique imaging appearances are secondary to 'Flip-Flop' phenomenon owing to underlying nutritional status of the patient. 'Flip-Flop' phenomenon on the Magnetic Resonance Imaging is result of an underlying pathological process of serous atrophy of bone marrow. Appreciation and recognition of this phenomenon will help in the correct interpretation of the images and leads a clinician toward appropriate management.


Assuntos
Medula Óssea/diagnóstico por imagem , Medula Óssea/patologia , Erros de Diagnóstico , Imageamento por Ressonância Magnética , Idoso , Anorexia Nervosa/complicações , Atrofia/diagnóstico por imagem , Atrofia/patologia , Caquexia/complicações , Feminino , Humanos , Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/patologia
12.
Nat Commun ; 12(1): 4559, 2021 07 27.
Artigo em Inglês | MEDLINE | ID: mdl-34315896

RESUMO

Activating mutations in the BRAF-MAPK pathway have been reported in histiocytoses, hematological inflammatory neoplasms characterized by multi-organ dissemination of pro-inflammatory myeloid cells. Here, we generate a humanized mouse model of transplantation of human hematopoietic stem and progenitor cells (HSPCs) expressing the activated form of BRAF (BRAFV600E). All mice transplanted with BRAFV600E-expressing HSPCs succumb to bone marrow failure, displaying myeloid-restricted hematopoiesis and multi-organ dissemination of aberrant mononuclear phagocytes. At the basis of this aggressive phenotype, we uncover the engagement of a senescence program, characterized by DNA damage response activation and a senescence-associated secretory phenotype, which affects also non-mutated bystander cells. Mechanistically, we identify TNFα as a key determinant of paracrine senescence and myeloid-restricted hematopoiesis and show that its inhibition dampens inflammation, delays disease onset and rescues hematopoietic defects in bystander cells. Our work establishes that senescence in the human hematopoietic system links oncogene-activation to the systemic inflammation observed in histiocytic neoplasms.


Assuntos
Senescência Celular , Hematopoese/genética , Células-Tronco Hematopoéticas/metabolismo , Histiocitose/patologia , Inflamação/patologia , Células Mieloides/patologia , Oncogenes , Animais , Medula Óssea/patologia , Pontos de Checagem do Ciclo Celular/genética , Senescência Celular/genética , Doença Crônica , Inibidor p16 de Quinase Dependente de Ciclina/metabolismo , Regulação da Expressão Gênica , Proteínas de Fluorescência Verde/metabolismo , Histiocitose/complicações , Humanos , Inflamação/complicações , Lentivirus/genética , Camundongos , Mutação/genética , Comunicação Parácrina , Análise de Componente Principal , Proteínas Proto-Oncogênicas B-raf/genética , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Fator de Necrose Tumoral alfa/metabolismo
13.
Int J Mol Sci ; 22(13)2021 Jun 25.
Artigo em Inglês | MEDLINE | ID: mdl-34202238

RESUMO

Acute myeloid leukemia (AML) is a hematologic malignancy caused by a wide range of alterations responsible for a high grade of heterogeneity among patients. Several studies have demonstrated that the hypoxic bone marrow microenvironment (BMM) plays a crucial role in AML pathogenesis and therapy response. This review article summarizes the current literature regarding the effects of the dynamic crosstalk between leukemic stem cells (LSCs) and hypoxic BMM. The interaction between LSCs and hypoxic BMM regulates fundamental cell fate decisions, including survival, self-renewal, and proliferation capacity as a consequence of genetic, transcriptional, and metabolic adaptation of LSCs mediated by hypoxia-inducible factors (HIFs). HIF-1α and some of their targets have been associated with poor prognosis in AML. It has been demonstrated that the hypoxic BMM creates a protective niche that mediates resistance to therapy. Therefore, we also highlight how hypoxia hallmarks might be targeted in the future to hit the leukemic population to improve AML patient outcomes.


Assuntos
Medula Óssea/metabolismo , Medula Óssea/patologia , Hipóxia/metabolismo , Leucemia Mieloide Aguda/etiologia , Leucemia Mieloide Aguda/metabolismo , Microambiente Tumoral , Animais , Linhagem Celular Tumoral , Reprogramação Celular , Gerenciamento Clínico , Suscetibilidade a Doenças , Metabolismo Energético , Epigênese Genética , Regulação Leucêmica da Expressão Gênica , Humanos , Fator 1 Induzível por Hipóxia/metabolismo , Leucemia Mieloide Aguda/patologia , Leucemia Mieloide Aguda/terapia , Terapia de Alvo Molecular , Células-Tronco Neoplásicas/metabolismo , Transdução de Sinais
14.
Int J Mol Sci ; 22(13)2021 Jun 22.
Artigo em Inglês | MEDLINE | ID: mdl-34206393

RESUMO

BACKGROUND: Chronic inflammation has been recognized in neoplastic disorders, including myeloproliferative neoplasm (MPN), as an important regulator of angiogenesis. AIMS: We investigated the influence of vascular endothelial growth factor (VEGF) and pro-inflammatory interleukin-6 (IL-6) on the expression of angiogenic factors, as well as inflammation-related signaling in mononuclear cells (MNC) of patients with MPN and JAK2V617F positive human erythroleukemic (HEL) cells. RESULTS: We found that IL-6 did not change the expression of angiogenic factors in the MNC of patients with MPN and HEL cells. However, IL-6 and the JAK1/2 inhibitor Ruxolitinib significantly increased angiogenic factors-endothelial nitric oxide synthase (eNOS), VEGF, and hypoxia-inducible factor-1 alpha (HIF-1α)-in patients with polycythemia vera (PV). Furthermore, VEGF significantly increased the expression of HIF-1α and eNOS genes, the latter inversely regulated by PI3K and mTOR signaling in the MNC of primary myelofibrosis (PMF). VEGF and inhibitors of inflammatory JAK1/2, PI3K, and mTOR signaling reduced the eNOS protein expression in HEL cells. VEGF also decreased the expression of eNOS and HIF-1α proteins in the MNC of PMF. In contrast, VEGF increased eNOS and HIF-1α protein expression in the MNC of patients with PV, which was mediated by the inflammatory signaling. VEGF increased the level of IL-6 immunopositive MNC of MPN. In summary, VEGF conversely regulated gene and protein expression of angiogenic factors in the MNC of PMF, while VEGF increased angiogenic factor expression in PV mediated by the inflammation-related signaling. CONCLUSION: The angiogenic VEGF induction of IL-6 supports chronic inflammation that, through positive feedback, further promotes angiogenesis with concomitant JAK1/2 inhibition.


Assuntos
Transtornos Mieloproliferativos/etiologia , Transtornos Mieloproliferativos/metabolismo , Neovascularização Patológica/genética , Neovascularização Patológica/metabolismo , Transdução de Sinais , Fator A de Crescimento do Endotélio Vascular/metabolismo , Biomarcadores , Medula Óssea/metabolismo , Medula Óssea/patologia , Linhagem Celular Tumoral , Citocinas/metabolismo , Regulação da Expressão Gênica , Humanos , Inflamação/etiologia , Inflamação/metabolismo , Mediadores da Inflamação/metabolismo , Janus Quinase 2/genética , Janus Quinase 2/metabolismo , Mutação , Transtornos Mieloproliferativos/patologia
15.
Molecules ; 26(11)2021 May 25.
Artigo em Inglês | MEDLINE | ID: mdl-34070537

RESUMO

INTRODUCTION: Radiotracers are widely used in medical imaging, using techniques of gamma-camera imaging (scintigraphy and SPECT) or positron emission tomography (PET). In bone marrow infection, there is no single routine test available that can detect infection with sufficiently high diagnostic accuracy. Here, we review radiotracers used for imaging of bone marrow infection, also known as osteomyelitis, with a focus on why these molecules are relevant for the task, based on their physiological uptake mechanisms. The review comprises [67Ga]Ga-citrate, radiolabelled leukocytes, radiolabelled nanocolloids (bone marrow) and radiolabelled phosphonates (bone structure), and [18F]FDG as established radiotracers for bone marrow infection imaging. Tracers that are under development or testing for this purpose include [68Ga]Ga-citrate, [18F]FDG, [18F]FDS and other non-glucose sugar analogues, [15O]water, [11C]methionine, [11C]donepezil, [99mTc]Tc-IL-8, [68Ga]Ga-Siglec-9, phage-display selected peptides, and the antimicrobial peptide [99mTc]Tc-UBI29-41 or [68Ga]Ga-NOTA-UBI29-41. CONCLUSION: Molecular radiotracers allow studies of physiological processes such as infection. None of the reviewed molecules are ideal for the imaging of infections, whether bone marrow or otherwise, but each can give information about a separate aspect such as physiology or biochemistry. Knowledge of uptake mechanisms, pitfalls, and challenges is useful in both the use and development of medically relevant radioactive tracers.


Assuntos
Medula Óssea/patologia , Compostos Radiofarmacêuticos/química , Humanos , Tomografia por Emissão de Pósitrons/métodos , Tomografia Computadorizada de Emissão de Fóton Único/métodos
17.
J Clin Exp Hematop ; 61(2): 109-113, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34092711

RESUMO

Thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly comprise TAFRO syndrome, which was proposed as a distinct clinical entity from iMCD without TAFRO syndrome (iMCD-NOS) due to its aggressive clinical course, refractoriness to corticosteroids, presence of thrombocytopenia, increased level of alkaline phosphatase, and normal level of gammaglobulin. However, diagnosing TAFRO syndrome in its early stages is challenging because it is rare and its diagnostic criteria are complicated. We describe a patient with TAFRO syndrome and adrenal hemorrhage who demonstrated a rapid decline in her clinical condition and did not respond to steroid pulse therapy, resulting in a fatal outcome. In the early stage of her clinical course, she developed unilateral adrenal hemorrhage with mild thrombocytopenia and normal clotting times, suggesting adrenal hemorrhage as a unique manifestation of TAFRO syndrome. In general, patients with TAFRO syndrome exhibit a more aggressive clinical course and poorer outcome than those with iMCD-NOS. To ameliorate this poor prognosis, it is important to diagnose the disease early and immediately start powerful immunosuppressive agents such as tocilizumab. Based on this case, adrenal hemorrhage may suggest TAFRO syndrome, and facilitate the rapid diagnosis of this complicated and rare disease.


Assuntos
Glândulas Suprarrenais/patologia , Hiperplasia do Linfonodo Gigante/complicações , Hemorragia/complicações , Idoso , Medula Óssea/patologia , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/patologia , Hiperplasia do Linfonodo Gigante/terapia , Feminino , Hemorragia/diagnóstico , Hemorragia/patologia , Hemorragia/terapia , Humanos
19.
J Ayub Med Coll Abbottabad ; 33(2): 335-338, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34137556

RESUMO

Bone-marrow being a home to various kinds of normal hematopoietic cells, sometime becomes overcrowded by abnormal cell population in malignancies like in acute myeloid leukaemia. One such dilemma in diagnosis betides when two abnormal cell populations in bone-marrow occur at the same time. A prime example is when reactive plasmacytosis in bone-marrow eventuate in relation with acute myeloid leukaemia (AML). Due to scarce amount of such cases reported, it is imperative to understand the difference between reactive plasmacytosis which arises after induction of chemotherapy and the one which is diagnosed along with AML, during initial diagnosis due to other causes, like infections and IL-6 production by the leukemic blast population. To substantiate these erstwhile arguments, the brief case history of a 45 years old female patient diagnosed with acute myeloid leukaemia with coincident reactive plasmacytosis having no previous history of chemotherapy is presented along with review of past published literature.


Assuntos
Medula Óssea/patologia , Leucemia Mieloide Aguda/diagnóstico , Feminino , Humanos , Leucemia Mieloide Aguda/patologia , Pessoa de Meia-Idade
20.
J Hematol Oncol ; 14(1): 87, 2021 06 04.
Artigo em Inglês | MEDLINE | ID: mdl-34088334

RESUMO

Cases of thrombotic thrombocytopenia induced by coronavirus disease 2019 (COVID-19) vaccines have been reported recently. Herein, we describe the first case of another critical disorder, hemophagocytic lymphohistiocytosis (HLH), in a healthy individual after COVID-19 vaccination. A 43-year-old Chinese farmer developed malaise, vomiting, and persistent high fever (up to 39.7 °C) shortly after receiving the first dose of the inactivated SARS-CoV-2 vaccine. The initial evaluation showed pancytopenia (neutrophil count, 0.70 × 109/L; hemoglobin, 113 g/L; platelet, 27 × 109/L), elevated triglyceride (2.43 mmol/L), and decreased fibrinogen (1.41 g/L). Further tests showed high serum ferritin levels (8140.4 µg/L), low NK cell cytotoxicity (50.13%-60.83%), and positive tests for Epstein-Barr virus (EBV) DNA. Hemophagocytosis was observed in the bone marrow. Therefore, HLH was confirmed, and dexamethasone acetate (10 mg/day) was immediately prescribed without etoposide. Signs and abnormal laboratory results resolved gradually, and the patient was discharged. HLH is a life-threatening hyperinflammatory syndrome caused by aberrantly activated macrophages and cytotoxic T cells, which may rapidly progress to terminal multiple organ failure. In this case, HLH was induced by the COVID-19 vaccination immuno-stimulation on a chronic EBV infection background. This report indicates that it is crucial to exclude the presence of active EBV infection or other common viruses before COVID-19 vaccination.


Assuntos
Vacinas contra COVID-19/efeitos adversos , COVID-19/prevenção & controle , Linfo-Histiocitose Hemofagocítica/etiologia , Adulto , Anti-Inflamatórios/uso terapêutico , Medula Óssea/patologia , Dexametasona/análogos & derivados , Dexametasona/uso terapêutico , Humanos , Linfo-Histiocitose Hemofagocítica/sangue , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/tratamento farmacológico , Masculino , SARS-CoV-2/isolamento & purificação
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