scholarly journals Hemophagocytic Lymphohistiocytosis in Autopsied Adults: Clinical, Laboratory and Immunohistochemical Evaluation for CD68 and CD57. Case Report and Literature Review

2019 ◽  
Vol 21 (4) ◽  
pp. 422
Author(s):  
Márcus Otávio Silva de Campos Menêses ◽  
Renata Margarida Etchebehere ◽  
Márcia Fernandes De Araújo ◽  
Ana Cristina Da Rocha Duque ◽  
Denise Bertulucci Rocha Rodrigues ◽  
...  

AbstractHemophagocytic lymphohistiocytosis (HLH) is a rare, usually fatal and underdiagnosed autoimmune-activated disease. The present study aimed to perform a macroscopic, histopathological and immunohistochemical evaluation for CD68 and CD57 in organs of autopsied adults with HLH. A total of 604 autopsy reports were analyzed, and all the patients that filled the diagnostic criteria for HLH (n = 2) were selected. These patients were 18 and 37 years old. Were evaluated both clinical and autopsy reports and performed histopathological and immunohistochemical analysis of the liver and spleen. Both patients filled the diagnostic criteria for HLH, as well as presented common signs and symptoms of this disease, such as chills, abdominal pain, diaphoresis, and jaundice. Hemophagocytosis was observed in the spleen, bone marrow, and lymph nodes of the two patients at autopsy. Immunostaining in the liver and spleen of both patients was mainly severe for CD68, and predominantly mild for CD57, indicating a decrease in NKC numbers and an increase in the number of macrophages, respectively. This was the first study to evaluate CD57 and CD68 in autopsies of adults with HLH. Thus, more studies are required, not only to better elucidate the pathogenetic mechanisms involved in the secondary HLH, but also to disseminate the results in the clinical environment, contributing to the early diagnosis and treatment with consequent reduction of mortality rate. Keywords: Autoimmune Diseases. Histiocytosis. Biomarkers. ResumoA Linfohistiocitose Hemofagocítica (HLH) é uma doença autoimune rara, geralmente fatal e subdiagnosticada. Este estudo tem como objetivo realizar avaliação macroscópica, histopatológica e imunohistoquímica para CD68 e CD57 em órgãos de pacientes adultos com HLH submetidos a autópsia. Um total de 604 laudos de autópsias foram analisados e todos os pacientes que preencheram os critérios diagnósticos para HLH (n = 2) foram selecionados. Esses pacientes tinham 18 e 37 anos de idade. Foram analisados tanto os prontuários quanto os laudos de autópsia, bem como foram realizadas análises histopatológicas e imunohistoquímicas do fígado e baço dos pacientes. Ambos preencheram os critérios diagnósticos para HLH e apresentarem sinais e sintomas comuns da doença, como calafrios, dor abdominal, sudorese e icterícia. A hemofagocitose foi observada no baço, medula óssea e linfonodos dos dois pacientes na autópsia. A imunohistoquímica do fígado e do baço de ambos os pacientes demonstrou imunomarcação acentuada para CD68 e predominantemente discreta para CD57, que indicam diminuição do número de NKC e aumento do número de macrófagos, respectivamente. Este foi o primeiro estudo a avaliar o CD57 e CD68 em autópsias de adultos com HLH. Assim, mais estudos são necessários, não apenas para melhor elucidar os mecanismos patogenéticos envolvidos na HLH secundária, mas também para disseminar os resultados no ambiente clínico, contribuindo para o diagnóstico e tratamento precoces com consequente redução da taxa de mortalidade. Palavras-chave: Doenças Autoimunes. Histiocitose. Biomarcadores.

Author(s):  

Acute leukemias are considered neoplasias that reach the hematopoietic system, resulting in rapid clonal expansion and accelerated proliferation of blood cells. In this way, the cells lose their capacity for maturation and differentiation, accumulating young cells (blasts) and without function in the bone marrow. Acute lymphocytic leukemia (ALL) is a neoplasm that affects cells of the lymphoid lineage, but its prevalence occurs in B-cell precursors compared to T cells. It is common to occur in children between 1 and 5 years of age, comprising about 80% of the cases of all leukemias in this age group, but in adults, the probability of being present is lower. The present study aimed at the accomplishment of a case report on acute lymphoid leukemia in children, based on the comparison of risk factors in the family, and the evaluation of socioeconomic interference in the treatment, where, through an explanatory booklet, health professionals and the community about the major signs and symptoms of ALL in children. The research was submitted to the Ethics Committee of the Catholic University Center of Quixadá, through the Brazil Platform where it was analyzed and approved (01673218.2.0000.5046). In this study, we reported the case of two twin sisters who entered the Hospital São Sebastião de Pedra Branca, Ceará at different times, where one was admitted with fever and boulders, and another presenting only fever, after laboratory tests they were referred to a referral hospital in Fortaleza, Ceará, and were then diagnosed with acute lymphoid leukemia. Due to the poor prognosis of this pathology and a possible worsening of the patients' clinical status, they were referred for bone marrow transplantation. Fortunately, after the treatments performed, the twin had a positive evolution, no longer needing to undergo bone marrow transplantation


2021 ◽  
pp. 000313482110474
Author(s):  
Yeahwa Hong ◽  
Christopher Staniorski ◽  
Dean Pollack ◽  
Steven Evans

Esophageal and gastric mucosal injuries are well-documented adverse effects of doxycycline leading to odynophagia, chest pain, and abdominal pain. There are no clear diagnostic criteria for such adverse effects; hence, the diagnosis depends heavily on thorough history. There is a paucity of literature describing life-threatening complications from doxycycline-induced mucosal injury, such as hemorrhage and perforation. We present the first case report describing a gastric perforation from doxycycline use.


2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
Michael Gilbertson ◽  
Annabel Tuckfield ◽  
Surender Juneja

We present the case of a 70-year-old woman who had a bone marrow examination performed to investigate marked thrombocytopenia in the context of a recent history of metastatic glucagonoma. Surprisingly this identified marked dysmegakaryopoiesis and fulfilled diagnostic criteria for refractory cytopenia with multilineage dysplasia, with a relatively uncommon associated cytogenetic lesion t(1;7). We present the case and review the literature of this cytogenetic lesion.


2020 ◽  
Vol 8 (11) ◽  
pp. 836-839
Author(s):  
E. Lemrabott ◽  
◽  
B. Mohamed Mahmoud ◽  
K. Mohammed Saoud ◽  
N. Mamouni ◽  
...  

The authors present an atypical case of complete hydatidiform mole.Woman, 27years old, who went to the emergency room for abdominal pain associated with 10 week pregnancy. Echography revealed the presence of hyper stimulated ovaries leading to diagnosisof spontaneous Ovarian Hyperstimulation Syndrome (OHSS). Blood sample revealed high levels of hCG (300000 U/L), which associated with the ultrasound finding of placenta with vesicular areas, led to the suspicion of CHM,Dilatation and evacuation (D&E) were performed at 11 weeks of gestation. The signs and symptoms of OHSS were the severest on day 4 after D&E, when hCGhad already decreased We must be aware that OHSS can occur duringmolar pregnancy, and can be exacerbated after D&E.


PEDIATRICS ◽  
1990 ◽  
Vol 85 (2) ◽  
pp. 215-217
Author(s):  
TSUNEZO SHIOTA ◽  
YUKIO YOSHIDA ◽  
SACHIYO HIRAI ◽  
SHOZO TORII

Intestinal myiasis occurs when fly eggs or larvae that were previously deposited in food are ingested and survive in the gastrointestinal tract. Some infested patients are asymptomatic; others have abdominal pain, vomiting, and diarrhea. Although babies and small children seem more susceptible to intestinal myiasis than adults, probably the age distribution can be explained by childhood activity, fearlessness of wormlike creatures, and experimentation.1,2 The signs and symptoms of intestinal myiasis can easily be confused with other intestinal disturbances. In this article, we describe the clinical course of intestinal myiasis in a 4-year-old boy with severe abdominal pain. CASE REPORT The patient was a 4-year-old previously healthy boy residing in Osaka City, Japan.


Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5274-5274
Author(s):  
Andres L. Brodsky ◽  
Brenner Sabando Velez ◽  
Curutchet Ragusin

Abstract Abstract 5274 Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a chronic and life-threatening hematopoietic stem cell disorder characterized by deficiency of the GPI-anchored complement inhibitory proteins CD55 and CD59 on blood cells. The resulting uncontrolled complement activation is responsible for chronic hemolysis and can lead to serious clinical morbidities including thromboembolism (TE) and chronic kidney disease (CKD), which have been shown to increase risk of mortality. Patients may also experience debilitating quality-of-life (QoL) issues, including fatigue, shortness of breath, erectile dysfunction, and abdominal pain, attributed to chronic hemolysis and resultant nitric oxide scavenging by free hemoglobin. Although hematopoietic stem cell transplantation (HSCT) remains the only potentially curative option for PNH, the risk for substantial morbidities and mortality still exist. In patients with PNH undergoing HSCT, up to 45% die or develop acute or chronic graft-versus-host disease. Eculizumab, a first-in-class terminal complement inhibitor, is the only approved treatment for patients with PNH. By inhibiting terminal complement activity and chronic hemolysis, eculizumab reduces the incidence of TE, CKD, and transfusion requirements, improves anemia and QoL, and normalizes survival in patients with PNH. Aim: Report the benefits of eculizumab in a patient with PNH who relapsed after HSCT. Case Report: A 27-year-old woman presented in December 1993 with fever, diarrhea, hemoglobinuria, and acute renal failure requiring temporary hemodialysis (Table). She was subsequently diagnosed with PNH. In February 1995, allogeneic HSCT from an HLA-identical sibling donor was performed. In 2003, 8 years after successful engraftment, the patient relapsed and presented with hemoglobinuria, abdominal pain, corticosteroid dependence requiring 20 to 40 mg methylprednisone, and high transfusional requirements. In November 2007, she had a granulocyte clone size of 37.2% as determined by flow cytometry. In June 2009, she started eculizumab therapy which was associated with a rapid reduction in lactate dehydrogenase (LDH) from 4964U/L to 456U/L. She subsequently achieved resolution of asthenia, disabling fatigue, and abdominal pain, as well as transfusion independence and improvement in hemoglobin. In May 2010, she had a granulocyte clone size of 86.1% as determined by both CD55- and CD59-negative cells, and a granulocyte clone size of 98.7% as determined by CD16b-negative cells. Conclusions: The only potential cure for PNH—bone marrow transplantation—is associated with high risks of morbidity and mortality; therefore, for most patients the associated risks preclude this option. In this case study, we show that HSCT may not be curative in all patients and the PNH associated symptoms can arise after BMT. The potential benefits of eculizumab in this patient population should be considered in light of recent data that demonstrated normalized survival in PNH patients receiving long-term eculizumab treatment. Disclosures: Brodsky: Alexion Pharmaceuticals, Inc.: Consultancy, Speakers Bureau. Velez:Alexion Pharmaceuticals, Inc.: Consultancy.


2017 ◽  
Vol 4 (1) ◽  
Author(s):  
Eimear Kitt ◽  
Kristina R. Brannock ◽  
Lauren A. VonHolz ◽  
Paul J. Planet ◽  
Erin Graf ◽  
...  

Abstract An 8-year old girl presented to our facility with a 10-day history of fever, fatigue, abdominal pain and refusal to walk. She recently travelled from her native Algeria where she first developed symptoms. On evaluation, she was ill-appearing, febrile and tachycardic with hepatosplenomegaly and lymphadenopathy noted on examination. A strong musty odor was also noted from the child. Laboratory evaluation revealed pancytopenia, hyponatremia, and an elevated AST, ALT, and LDH. Malaria testing was negative, as was a PPD. On further questioning, the family reported multiple sick contacts in Algeria with similar symptoms. After discussion with Oncology and Infectious Diseases, she underwent a bone marrow biopsy that was significant for multiple non-caseating ring granulomas. She was started on combination therapy of doxycycline and for presumed brucellosis infection with improvement in her symptoms and resolution of fever. Bone marrow culture returned several days later positive for Brucella melitensis.


2020 ◽  
Vol 78 (4) ◽  
pp. 369-372
Author(s):  
Virgínia Vinha Zanuncio ◽  
Kelvin Oliveira Rocha

Dyskeratosis Congenita (DC) is a hereditary and multisystemic syndrome, with heterogeneous clinical and genetic manifestations, characterized as a disease associated with maintenance of defects and early telomere shortening. It is a rare condition, with an estimated annual incidence of 1 in 1 million individuals, and is more common in males than females, with an approximate ratio of 10:1. The main initial clinical manifestations are disorders of cutaneous pigmentation, nail dystrophy and abnormalities in the oropharynx, usually occurring between five and twelve years of age. Bone marrow failure (BMF) is the leading cause of death related to this disease. We present a case report of a child, female, two years old, who presented the cardinal signs and symptoms of DC at an early age, and we emphasize the importance of multidisciplinary monitoring of the patient.


2020 ◽  
Vol 90 (5) ◽  
pp. 535-541
Author(s):  
Alan Maksimović ◽  
◽  
Dajna Preldžić ◽  
Ismar Lutvikadić ◽  
Amir Zahirović ◽  
...  

There are only a few reported cases of abdominal ectopic pregnancy and consequential foetal mummification in dogs. The majority of the described cases indicate that this condition may exist for a prolonged period of time without any manifestation of illness. Therefore, sudden signs of abdominal distension, abdominal pain, episodes of epileptiform seizures, dark diarrhoea and lethargy, as were observed in the present case, are not likely to be considered as symptoms of an abdominal ectopic pregnancy. Furthermore, to the best of our knowledge, this is the first report of an eight-year long abdominal pregnancy in an ovariohysterectomised bitch. We present the clinical, laboratory, radiological and intraoperative findings, and the medical and surgical therapy for an abdominal ectopic pregnancy in a 14 year old Miniature Pinscher bitch, ovariohysterectomised eight years previously.


2004 ◽  
Vol 128 (3) ◽  
pp. 321-323 ◽  
Author(s):  
Ding Wang ◽  
Muhammad S. Shurafa ◽  
Radhika Acharya ◽  
Vernon F. Strand ◽  
Michael D. Linden

Abstract Heterotopic ossification is rarely seen after midline abdominal surgery. The etiology of heterotopic ossification is unknown. Although it is well recognized that heterotopic ossification may contain osteogenic cells and/or hematopoietic cells, to our knowledge, no case has ever been reported to have histologic evidence of hematopoiesis. We report the occurrence of heterotopic bone with bone marrow showing normal trilineage hematopoiesis in the incision scar of a woman who underwent gastric reduction surgery for the treatment of obesity. The literature regarding heterotopic ossification and extramedullary hematopoiesis is reviewed in this report, and discussion focuses on the mechanism of this pathophysiologic process.


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