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JAMA Dermatology Jan 2019Pyoderma gangrenosum and necrotizing Sweet syndrome are diagnostically challenging variants of neutrophilic dermatosis that can clinically mimic the cutaneous and...
IMPORTANCE
Pyoderma gangrenosum and necrotizing Sweet syndrome are diagnostically challenging variants of neutrophilic dermatosis that can clinically mimic the cutaneous and systemic features of necrotizing fasciitis. Improved characterization of these rare variants is needed, as improper diagnosis may lead to inappropriate or delayed treatment and the potential for morbidity.
OBJECTIVE
To determine the characteristics of necrotizing neutrophilic dermatosis to improve diagnostic accuracy and distinguish from infection.
DESIGN, SETTING, AND PARTICIPANTS
A case series of patients with necrotizing neutrophilic dermatosis treated at 3 academic hospitals (University of California San Francisco, Oregon Health and Science University, and University of Minnesota) from January 1, 2015, to December 31, 2017, was performed along with a literature review of related articles published between January 1, 1980, and December 31, 2017. Data were obtained from medical records as well as Medline and Embase databases. All patients had signs resembling necrotizing infection and had a final diagnosis of pyoderma gangrenosum with systemic features or necrotizing Sweet syndrome. Patients were excluded if a diagnosis other than neutrophilic dermatosis was made, if key clinical information was missing, and if reported in a non-English language.
MAIN OUTCOMES AND MEASURES
Description of key characteristics of necrotizing neutrophilic dermatosis.
RESULTS
Overall, 54 patients with necrotizing neutrophilic dermatosis were included, of which 40 had pyoderma gangrenosum with systemic features and 14 had necrotizing Sweet syndrome. Of the 54 patients, 29 (54%) were male and 25 (46%) were female, with a mean (SD) age of 51 (19) years. Skin lesions commonly occurred on the lower (19 [35%]) and upper (13 [24%]) extremities and developed after a surgical procedure (22 [41%]) or skin trauma (10 [19%]). Shock was reported in 14 patients (26%), and leukemoid reaction was seen in 15 patients (28%). Of the patients with necrotizing neutrophilic dermatosis, 51 (94%) were initially misdiagnosed as necrotizing fasciitis and subsequently received inappropriate treatment. Debridement was performed in 42 patients (78%), with a mean (SD) of 2 (2 [range, 1-12]) debridements per patient. Four amputations (7%) were performed. Forty-nine patients (91%) received antibiotics when necrotizing neutrophilic dermatosis was misdiagnosed as an infection, and 50 patients (93%) received systemic corticosteroids; all patients responded to immunosuppressants.
CONCLUSIONS AND RELEVANCE
A complex spectrum of clinical findings of pyoderma gangrenosum and Sweet syndrome with prominent systemic inflammation exists that defines a new subset of neutrophilic dermatoses, termed necrotizing neutrophilic dermatoses; recognizing the difference between this variant and severe infection may prevent unnecessary surgical procedures and prolonged disease morbidity associated with a misdiagnosis and may expedite appropriate medical management.
Topics: Diagnosis, Differential; Fasciitis, Necrotizing; Female; Humans; Immunosuppressive Agents; Male; Middle Aged; Neutrophils; Prognosis; Retrospective Studies; Skin; Sweet Syndrome
PubMed: 30383110
DOI: 10.1001/jamadermatol.2018.3890 -
Cureus Sep 2022Leukocytosis is defined by an increased WBC count in the peripheral blood. This can be caused by many pathologies from benign conditions such as stress, infection, and...
Leukocytosis is defined by an increased WBC count in the peripheral blood. This can be caused by many pathologies from benign conditions such as stress, infection, and inflammation or malignant origins such as leukemia. Although leukocytosis is regularly encountered clinically and has many etiologies making a definitive diagnosis, at times, may be difficult. A case of severe leukocytosis requires careful consideration of symptoms and confirmation with serial complete blood count (CBC) testing before pursuing further invasive testing such as bone marrow biopsy. Here, we report the case of a 78-year-old male patient who, after a cardiac arrest, presented with reactive hyperleukocytosis mimicking acute monocytic leukemia.
PubMed: 36299948
DOI: 10.7759/cureus.29508 -
Annals of Medicine and Surgery (2012) Apr 2023Leukemoid reaction (increase in leucocyte count >50 ×10 cell/l) occurs due to reactive causes of bone marrow and is diagnosed after excluding the malignant...
UNLABELLED
Leukemoid reaction (increase in leucocyte count >50 ×10 cell/l) occurs due to reactive causes of bone marrow and is diagnosed after excluding the malignant haematological disorder. Leukemoid reaction is a rare clinical presentation in metastatic renal cell carcinoma and is said to have a rare prognosis. This case has had been reported in the line of SCARE criteria.
CASE PRESENTATION
A case of a 35-year-old female with no known previous co-morbidities presented with a history of abdominal pain in the right flank region for 2 months, fever and cough for 2 months. Physical examination showed palpable mass and tenderness in the right flank and investigations showed leukemoid reaction in peripheral blood smear. The patient was initially treated with strong intravenous antibiotics with suspicion of pyelonephritis in another centre, despite which the patient still had elevated leucocyte count and referred to our centre, where the patient was evaluated for elevated leucocyte count and with further investigations, ruled out any malignant haematological disorder. Final diagnosis of renal cell carcinoma was made by renal mass biopsy. The patient underwent targeted therapy with sunitinib. The patient expired and further investigation and follow-up were not possible.
CONCLUSION
The lack of data and evidence of extensive diagnostic tests is the reason we are unable to assume leukemoid reaction as a poor prognostic factor in case of metastatic renal cell carcinoma. The presence of other paraneoplastic syndromes with renal cell carcinoma might have resulted in the poor prognosis that cannot be excluded.
PubMed: 37113841
DOI: 10.1097/MS9.0000000000000513 -
Hematology. American Society of... Dec 2022Children with Down syndrome (DS) have a greater than 100-fold increased risk of developing acute myeloid leukemia (ML) and an approximately 30-fold increased risk of...
Children with Down syndrome (DS) have a greater than 100-fold increased risk of developing acute myeloid leukemia (ML) and an approximately 30-fold increased risk of acute lymphoblastic leukemia (ALL) before their fifth birthday. ML-DS originates in utero and typically presents with a self-limiting, neonatal leukemic syndrome known as transient abnormal myelopoiesis (TAM) that is caused by cooperation between trisomy 21-associated abnormalities of fetal hematopoiesis and somatic N-terminal mutations in the transcription factor GATA1. Around 10% of neonates with DS have clinical signs of TAM, although the frequency of hematologically silent GATA1 mutations in DS neonates is much higher (~25%). While most cases of TAM/silent TAM resolve without treatment within 3 to 4 months, in 10% to 20% of cases transformation to full-blown leukemia occurs within the first 4 years of life when cells harboring GATA1 mutations persist and acquire secondary mutations, most often in cohesin genes. By contrast, DS-ALL, which is almost always B-lineage, presents after the first few months of life and is characterized by a high frequency of rearrangement of the CRLF2 gene (60%), often co-occurring with activating mutations in JAK2 or RAS genes. While treatment of ML-DS achieves long-term survival in approximately 90% of children, the outcome of DS-ALL is inferior to ALL in children without DS. Ongoing studies in primary cells and model systems indicate that the role of trisomy 21 in DS leukemogenesis is complex and cell context dependent but show promise in improving management and the treatment of relapse, in which the outcome of both ML-DS and DS-ALL remains poor.
Topics: Infant; Infant, Newborn; Child; Humans; Child, Preschool; Down Syndrome; Leukemoid Reaction; GATA1 Transcription Factor; Leukemia, Myeloid, Acute; Mutation
PubMed: 36485097
DOI: 10.1182/hematology.2022000395 -
Annales de Biologie Clinique 2012Leukemoid reaction is a rare paraneoplastic syndrome. It can occur in association with carcinomas, in particular of the lung, gastric and renal. However, its association...
Leukemoid reaction is a rare paraneoplastic syndrome. It can occur in association with carcinomas, in particular of the lung, gastric and renal. However, its association with sarcoma is infrequent. Leukemoid reaction occuring in patients with uterine sarcoma have not been previously reported. We report the case of a « leukemoid » leucocytosis revealing an uterine sarcoma with fatal evolution.
Topics: Fatal Outcome; Female; Humans; Leukemoid Reaction; Leukocytosis; Middle Aged; Sarcoma; Uterine Neoplasms
PubMed: 22565182
DOI: 10.1684/abc.2012.0712 -
Anaerobe Oct 2022Paeniclostridium sordellii is a pathogen that causes rapidly fatal infections characterized by severe edema, extreme leukemoid reaction and lack of an innate immune...
OBJECTIVE
Paeniclostridium sordellii is a pathogen that causes rapidly fatal infections characterized by severe edema, extreme leukemoid reaction and lack of an innate immune response. We recently identified a metalloproteinase of P. sordellii-1 (Mcs1) that cleaves human vascular cell adhesion molecule 1, an adhesion molecule important to hematopoietic precursor retention and leukocyte diapedesis. In the current study, we further characterize Mcs1 activity and investigate its role in pathogenesis.
METHODS
Mcs1 peptide cleavage sequence and activity conditions were identified using a semi-quantitative fluorescence-based reporter assay. Additional host targets for Mcs1 protease activity were tested and confirmed by gel electrophoreses and western blots. Finally, Mcs1 knock out (ΔMcs1) and complemented (cMcs1) strains were developed for assessment in our animal model of myonecrosis.
RESULTS
Data show that Mcs1 prefers aliphatic amino acid residues, I or L, especially when adjacent to negatively charged or noncharged-polar residues. In vitro, Mcs1 cleaved or partially cleaved human cell adhesion molecules, E-selectin and intracellular adhesion molecule-1 (ICAM-1), and mediators of innate immune infection defense, complement protein-3 and antimicrobial peptide LL-37. In vivo, infection with the ΔMcs1 P. sordellii strain had little effect on animal survival, tissue destruction or circulating white blood cell counts compared to wild type and cMcs1 strains.
CONCLUSIONS
Similar to proteolytic virulence factors from other pathogens, Mcs1 is a promiscuous protease that cleaves multiple human-host factors. Despite minimal impact of Mcs1 on the murine model of P. sordellii infection, it is worth considering its role in humans and other animal models.
Topics: Animals; Humans; Mice; Clostridium sordellii; Disease Models, Animal; Peptide Hydrolases; Virulence Factors; Clostridium Infections; Bacterial Proteins
PubMed: 34688909
DOI: 10.1016/j.anaerobe.2021.102468 -
Autopsy & Case Reports Jun 2020Hepatic cirrhosis, diabetes mellitus and iron overload can each independently predispose to cryptococcosis. Hereditary hemochromatosis leads to all three of these...
Hepatic cirrhosis, diabetes mellitus and iron overload can each independently predispose to cryptococcosis. Hereditary hemochromatosis leads to all three of these predispositions. This report is the case of a patient with chronic hepatitis B virus infection and cirrhosis, who had markedly elevated serum ferritin and 99% transferrin saturation, and developed a leukemoid reaction. Autopsy revealed disseminated cryptococcosis for which the leukemoid reaction was a clue and possible hereditary hemochromatosis of which elevated ferritin and transferrin saturation can be clues. Hereditary hemochromatosis is an important diagnosis clinicians should never miss because early treatment with phlebotomy can be life-saving. Disseminated cryptococcosis can be rapidly diagnosed with serum cryptococcal antigen test and is treatable.
PubMed: 33344301
DOI: 10.4322/acr.2020.180 -
Cureus May 2021Pulmonary malignancies are known to have high prevalence and mortality. They are associated with different paraneoplastic syndromes, especially pulmonary carcinomas,...
Pulmonary malignancies are known to have high prevalence and mortality. They are associated with different paraneoplastic syndromes, especially pulmonary carcinomas, because they are more common than pulmonary sarcomas. We present a case of a 56-year-old African American male who was admitted to our institution with a three-month history of a dry cough, progressive shortness of breath, and two to three days of right arm swelling. He had extreme leukocytosis (WBC count of 106,500 cells/mm). Computed tomography (CT) scan of the thorax demonstrated an irregular, thick-walled 14-cm lung mass occupying the middle and upper hemithorax. CT-guided biopsy of the mass confirmed the diagnosis of lung sarcoma.
PubMed: 34150398
DOI: 10.7759/cureus.15047 -
Cureus May 2023()and coronavirus disease 2019 (COVID-19) infections can have overlapping symptoms. Recently, the association and outcomes of coinfection have been studied. We present...
()and coronavirus disease 2019 (COVID-19) infections can have overlapping symptoms. Recently, the association and outcomes of coinfection have been studied. We present the case of an 83-year-old lady with Parkinson's disease (PD) who was admitted with pneumonia secondary to severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) infection. She was treated with empiric antibiotics ampicillin-sulbactam and azithromycin, along with antiviral therapy remdesivir and baricitinib, and dexamethasone. The patient developed severe infection with a leukemoid reaction. She was treated with intravenous metronidazole and oral vancomycin without any improvement. Before she could receive a fecal microbiota transplant, her infection progressed to fulminant colitis, and she required emergent surgery. The patient developed several complications post-surgery and succumbed to the severe illness. Our patient's multiple comorbidities and an underlying COVID-19 infection predisposed her to severe illness. This case emphasizes the long-standing discussion on antibiotic stewardship and encourages a debate on the role of immunosuppressant antiviral medications and underlying PD in predisposing patients to a severe infection.
PubMed: 37265903
DOI: 10.7759/cureus.38401 -
EJNMMI Research Feb 2020The undifferentiated embryonic sarcoma of the liver (UESL) is a rare, aggressive tumor mainly affecting children. Since UESL has no specific clinical symptoms or imaging...
BACKGROUND
The undifferentiated embryonic sarcoma of the liver (UESL) is a rare, aggressive tumor mainly affecting children. Since UESL has no specific clinical symptoms or imaging characteristics, many cases of UESL are diagnosed late. The paraneoplastic leukemoid reaction (PLR) is a very rare concomitant of oncological patients associated with poor prognosis. This report describes the clinical course of a patient combining these two rare entities and describes the diagnostic challenges and dynamics of paraneoplastic syndrome.
CASE PRESENTATION
We report a case of UESL in a 46-year-old male who became clinically conspicuous due to pronounced B symptoms. CT and MRI showed a suspicious unifocal liver lesion. As the histological analysis of a tissue sample did not reveal a clear result, an 18F-FDG-PET-CT examination was performed. In addition to a high glucose metabolism of the liver lesion, an increased glucose metabolism in the entire bone marrow was observed. This finding was considered as pronounced paraneoplasia and laparotomy with liver segment resection followed. Immediately after resection of the tumor the paraneoplastic symptoms completely declined and the patient showed no signs of recurrence in the 1-year follow-up.
CONCLUSIONS
Although UESL is rare and predominantly affects children, this diagnosis should always be considered for unclear unifocal cystic liver lesions, regardless of the patient's age, as appropriate treatment has a good prognosis.
PubMed: 32072333
DOI: 10.1186/s13550-020-0602-x