Kasabach-Merritt phenomenon with cellulitis in infant

2020 ◽  
Vol 13 (9) ◽  
pp. e235065
Author(s):  
Ariyati Yosi ◽  
Abdullah Afif Siregar ◽  
Yasmine Siregar ◽  
Widyaningsih Oentari
Keyword(s):  
Oral Corticosteroid ◽  
Vascular Lesion ◽  
Severe Thrombocytopenia ◽  
Laboratory Findings ◽  
Blood Examination ◽  
Normochromic Anaemia ◽  
Normocytic Normochromic Anaemia ◽  
Tufted Angioma ◽  
History Of ◽  
D Dimer

Kasabach-Merritt phenomenon (KMP) is consumptive coagulopathy in large vascular lesion. This condition is specific in kaposiform haemangioendothelioma (KHE) and tufted angioma, associated with high mortality rate. This condition often involves the limbs, trunk and retroperitoneum. The clinical features that are usually found are purpura, oedema, induration and ecchymosis. A boy baby aged 9 months presented with a history of violaceous tumour that appeared on his left leg at birth that became enlarged since 6 months ago that caused discrepancy between both of his legs. The patient has his left leg massaged through alternative treatment, causing it became bruised and wounded. Blood examination showed normocytic normochromic anaemia with severe thrombocytopenia and high D-dimer. The patient was diagnosed with KMP with cellulitis and treated with oral corticosteroid, propranolol and antibiotics. Diagnosis of KMP can be made through clinical and laboratory findings. Combination therapy with corticosteroid and propranolol can be considered for KMP treatment.

Thienopyridine-Associated Thrombotic Thrombocytopenia Purpura: Updated Antibody Results From the SERF-TTP Study.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 892-892
Author(s):  
Anaadriana Zakarija ◽  
Thanh Ha Luu ◽  
Hau C. Kwaan ◽  
June McKoy ◽  
Ivy Weiss ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Neutralizing Antibodies ◽  
Conflicts Of Interest ◽  
Therapeutic Plasma Exchange ◽  
Severe Thrombocytopenia ◽  
Adamts13 Activity ◽  
Laboratory Findings ◽  
History Of ◽  
Adamts13 Deficiency ◽  
Drug Dependent

Abstract Abstract 892 Background: The thienopyridines, ticlopidine and clopidogrel, have been associated with thrombotic thrombocytopenia purpura (TTP). However, few studies have reported information on antibodies to ADAMTS13 among patients with thienopyridine-associated TTP. We previously reported on two mechanistic pathways of thienopyridine-associated TTP with some overlapping features. Evaluation of ADAMTS13 autoantibodies was undertaken to improve understanding of these syndromes. Methods: Clinical and laboratory findings were evaluated for 30 ticlopidine-, 10 clopidogrel-associated TTP cases, and 54 cases of idiopathic TTP. Results: Among patients with thienopyridine-induced TTP, those with a history of ticlopidine versus clopidogrel use were more likely to present with severe thrombocytopenia (platelet < 20,000) (90% versus 13%), severe ADAMTS13-deficiency (80% versus 0%), and neutralizing antibodies to ADAMTS13 (100% versus 0%), and were less likely to have less than a two week history of thienopyridine exposure (0% versus 50%) (p<0.05 for each comparison). They were also more likely to survive following therapeutic plasma exchange (TPE) (85% versus 50%). 2 patients exposed to clopidogrel later relapsed and had similar characteristics to idiopathic TTP patients with non-deficient ADAMTS13 activity. Conclusion: Ticlopidine causes TTP by a pathway involving a neutralizing autoantibody to ADAMTS13 while clopidogrel causes TTP by an ADAMTS13-independent pathway. Although ADAMTS13 autoantibodies are present in both idiopathic and ticlopidine-associated TTP, spontaneous relapses are not seen in ticlopidine-associaated TTP, suggesting that drug-dependent antibodies are present. Clopidogrel associated TTP is distinct from idiopathic TTP in that ADAMTS13 autoantibodies are absent and response to TPE is poor. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Ehrlichia Meningoencephalitis: A Case Series

2021 ◽  
Vol 8 (2) ◽  
Author(s):  
Morena J ◽  
◽  
Antimisiaris M ◽  
Singh D ◽  
◽  
...  
Keyword(s):  
Elderly Patients ◽  
Direct Detection ◽  
Case Reports ◽  
Case Series ◽  
Suspected Case ◽  
Severe Thrombocytopenia ◽  
Laboratory Findings ◽  
Elevated Protein ◽  
History Of

Objective: We present the distinct clinical and laboratory findings in two cases of Ehrlichia meningoencephalitis, along with one suspected case. Background: The number of cases of Ehrlichia chaffeensis reported to the CDC has more than doubled from 2007-2017. A PubMed literature search using the words “Ehrlichiosis and meningoencephalitis” revealed five case reports with neurologic manifestations. Design/Methods: This is a retrospective observational study. Two elderly patients presented with encephalopathy, fever, transaminitis, thrombocytopenia, a positive E. chaffeensis Polymerase Chain Reaction (PCR) in Serum, and Cerebrospinal Fluid (CSF) with a lymphocytic or neutrophilic pleocytosis and elevated protein. One patient had similar symptoms and a positive E. chaffeensis PCR, but lumbar puncture was unable to be performed due to severe thrombocytopenia. They presented in May or June. Doxycycline was started within 2-3 days after presentation to the hospital. Follow up five months later revealed all patients were close, or back to baseline. Results and Conclusions: Suspicion of Ehrlichia meningoencephalitis should be raised in elderly patients presenting with fever and encephalopathy in the summer season with history of tick bite or residence in wooded areas. Thrombocytopenia and transaminitis should raise further suspicion. CSF studies typically show a lymphocytic pleocytosis and elevated protein. PCR technique allows for direct detection of pathogen-specific DNA and is the preferred method of detection during the acute phase of illness. Prompt treatment with doxycycline results in good outcomes. Doxycycline is not included in the typical meningitis regimen, therefore, this disease is important to quickly identify as delay in Doxycycline can result in worse outcomes.

Kasabach-Merritt Syndrome

2002 ◽  
Vol 6 (4) ◽  
pp. 335-339 ◽  
Author(s):  
Sheilagh Maguiness ◽  
Lyn Guenther
Keyword(s):  
Clinical Presentation ◽  
Treatment Guidelines ◽  
Case Reports ◽  
Treatment Options ◽  
Vascular Lesion ◽  
Vascular Pathology ◽  
Vascular Tumors ◽  
Kaposiform Hemangioendothelioma ◽  
Laboratory Findings ◽  
Tufted Angioma

Background: Kasabach-Merritt syndrome (KMS) is a consumptive coagulopathy associated with the presence of a large vascular lesion. It is often a frustrating condition to treat and it carries a high mortality rate. There are currently no known treatment guidelines. Kasabach-Merritt syndrome is associated with kaposiform hemangioendothelioma (KHE) and tufted angioma (TA); these lesions, when associated with KMS, are locally invasive, aggressive vascular tumors. Treatment options include supportive care, local therapies, and drug and surgical management. In most recent case reports, a multimodal approach to therapy is taken. Objective and Conclusion: The objective of this article is to provide a comprehensive review of KMS and give an up-to-date summary of treatment options. The clinical presentation, laboratory findings, vascular pathology, and pathophysiology will also be discussed.

Hepatic brucelloma: a rare complication of a common zoonotic disease

2020 ◽  
Vol 13 (12) ◽  
pp. e237076
Author(s):  
George Vatidis ◽  
Eirini I Rigopoulou ◽  
Konstantinos Tepetes ◽  
George N Dalekos
Keyword(s):  
Rare Complication ◽  
Surgical Excision ◽  
Coombs Test ◽  
Inflammation Markers ◽  
Laboratory Findings ◽  
Ct Guided ◽  
Rare Manifestation ◽  
Bone Marrow Cultures ◽  
History Of ◽  
The Right

Hepatic brucelloma (HB), a rare manifestation of brucellosis, refers to liver involvement in the form of abscess. A 35-year-old woman stockbreeder was admitted due to 1-month history of evening fever, sweating and weight loss, while she was on 3-week course of rifampicin/doxycycline for suspected brucellosis. On admission, she had hepatosplenomegaly and a systolic murmur, while cholestasis, increased inflammation markers and a strong-positive Wright-Coombs test were the main laboratory findings. As blood and bone marrow cultures were unrevealing, further investigation with CT imaging showed a central liver calcification surrounded by heterogeneous hypodense area being compatible with HB. Material from CT-guided drainage tested negative for Brucella spp. After failure to improve on a 10-week triple regiment, surgical excision was decided and Brucella spp were identified by PCR. Our case highlights challenges in establishing HB diagnosis, which should be considered on the right epidemiological context and when serological and radiological evidence favour its diagnosis.

scholarly journals Euglycemic Diabetic Ketoacidosis after a Single Dose of Empagliflozin in a Patient with Pancreatitis

2021 ◽  
Vol 11 (2) ◽  
pp. 216-218
Author(s):  
Marta Brandão Calçada ◽  
Luís Fernandes ◽  
Rita Soares Costa ◽  
Sara Montezinho ◽  
Filipa Martins Duarte ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Diabetic Ketoacidosis ◽  
Drug Class ◽  
Clinical Suspicion ◽  
Laboratory Findings ◽  
Sodium Glucose Cotransporter ◽  
History Of ◽  
High Degree ◽  
Euglycemic Diabetic Ketoacidosis

Sodium-glucose cotransporter 2 inhibitors (SGLT2i) are the most recently approved drug class for the treatment of type 2 diabetes mellitus (T2D). Although they are largely well-tolerated, their intake has been associated with euglycemic diabetic ketoacidosis (DKA) in some rare cases. We report the case of a 70-year-old male with type 2 diabetes and no history of DKA, who started therapy with empagliflozin one day before presenting with acute pancreatitis and laboratory findings consistent with euglycemic DKA. SGLT2i can induce euglycemic DKA from the first dose. Given the atypical presentation, a high degree of clinical suspicion is required to recognize this complication.

scholarly journals Severe Posaconazole-Induced Glucocorticoid Deficiency with Concurrent Pseudohyperaldosteronism: An Unfortunate Two-for-One Special

2021 ◽  
Vol 7 (8) ◽  
pp. 620
Author(s):  
Alejandro Villar-Prados ◽  
Julia J. Chang ◽  
David A. Stevens ◽  
Gary K. Schoolnik ◽  
Samantha X. Y. Wang
Keyword(s):  
Mechanism Of Action ◽  
Antifungal Agents ◽  
Laboratory Findings ◽  
Steroid Synthesis ◽  
Adrenal Steroid ◽  
History Of ◽  
Glucocorticoid Deficiency

A 56-year-old Hispanic man with a history of disseminated coccidioidomycosis was diagnosed with persistent glucocorticoid insufficiency and pseudohyperaldosteronism secondary to posaconazole toxicity. This case was notable for unexpected laboratory findings of both pseudohyperaldosteronism and severe glucocorticoid deficiency due to posaconazole’s mechanism of action on the adrenal steroid synthesis pathway. Transitioning to fluconazole and starting hydrocortisone resolved the hypokalemia but not his glucocorticoid deficiency. This case highlights the importance of recognizing iatrogenic glucocorticoid deficiency with azole antifungal agents and potential long term sequalae.

scholarly journals Atypical lymphoid cells circulating in blood in COVID-19 infection: morphology, immunophenotype and prognosis value

2020 ◽  
pp. jclinpath-2020-207087
Author(s):  
Anna Merino ◽  
Alexandru Vlagea ◽  
Angel Molina ◽  
Natalia Egri ◽  
Javier Laguna ◽  
...  
Keyword(s):  
T Cells ◽  
Cd8 T Cells ◽  
Automatic System ◽  
Lymphoid Cells ◽  
Effector Memory ◽  
Laboratory Findings ◽  
Deep Blue ◽  
Sensitivity Specificity ◽  
Reactive Lymphocytes ◽  
D Dimer

AimsAtypical lymphocytes circulating in blood have been reported in COVID-19 patients. This study aims to (1) analyse if patients with reactive lymphocytes (COVID-19 RL) show clinical or biological characteristics related to outcome; (2) develop an automatic system to recognise them in an objective way and (3) study their immunophenotype.MethodsClinical and laboratory findings in 36 COVID-19 patients were compared between those showing COVID-19 RL in blood (18) and those without (18). Blood samples were analysed in Advia2120i and stained with May Grünwald-Giemsa. Digital images were acquired in CellaVisionDM96. Convolutional neural networks (CNNs) were used to accurately recognise COVID-19 RL. Immunophenotypic study was performed throughflow cytometry.ResultsNeutrophils, D-dimer, procalcitonin, glomerular filtration rate and total protein values were higher in patients without COVID-19 RL (p<0.05) and four of these patients died. Haemoglobin and lymphocyte counts were higher (p<0.02) and no patients died in the group showing COVID-19 RL. COVID-19 RL showed a distinct deep blue cytoplasm with nucleus mostly in eccentric position. Through two sequential CNNs, they were automatically distinguished from normal lymphocytes and classical RL with sensitivity, specificity and overall accuracy values of 90.5%, 99.4% and 98.7%, respectively. Immunophenotypic analysis revealed COVID-19 RL are mostly activated effector memory CD4 and CD8 T cells.ConclusionWe found that COVID-19 RL are related to a better evolution and prognosis. They can be detected by morphology in the smear review, being the computerised approach proposed useful to enhance a more objective recognition. Their presence suggests an abundant production of virus-specific T cells, thus explaining the better outcome of patients showing these cells circulating in blood.

SURGERY IN A HEMOPHILIAC CHILD WITH INTRACRANIAL HEMORRHAGE

PEDIATRICS ◽  
1961 ◽  
Vol 28 (5) ◽  
pp. 800-804
Author(s):  
John H. Fuerth ◽  
Paul Teng ◽  
Erwin Goldenberg
Keyword(s):  
Los Angeles ◽  
Type A ◽  
Bleeding Tendency ◽  
Laboratory Findings ◽  
Excessive Bleeding ◽  
Surgical Interventions ◽  
Fatal Hemorrhage ◽  
History Of ◽  
System 2 ◽  
The Central Nervous System

THE UNUSUAL bleeding tendency in hemophiliacs has been known since biblical times, and its hazards have been recognized in even such simple surgical procedures as circumcision.1 Perhaps the most dangerous complication of hemophilia is bleeding into the central nervous system.2 It therefore seems worthwhile to report the case of a 2-year-old hemophiliac who survived several intracranial hemorrhages, with two surgical interventions, but who 4 months later had a fourth and fatal hemorrhage. CASE REPORT History D. H. was a 2-year-old hemophiliac with numerous admissions to the Kaiser Foundation Hospital, Los Angeles, for bleeding episodes. He was born at another hospital, was circumcised shortly after birth and had excessive bleeding following this. His first admission was at the age of 1 year for bleeding following a tongue bite. At that time he had an abnormal result of a prothrombin consumption test, with 55% residual prothrombin in the serum. The prothrombin consumption was corrected by fresh normal plasma and barium sulfate adsorbed plasma, but not by serum or plasma from a known hemophiliac type A. The diagnosis of hemophilia type A was thus established. He had two brothers who were investigated and found to be normal. There was no abnormal bleeding tendency on his father's side, but two uncles of his mother and two first cousins were said to be "bleeders." No further details were known. Physical and Laboratory Findings The boy was admitted to the hospital on the evening of October 13, 1957, with a history of vomiting and progressive lethargy of 2 days' duration.

A Cross-Sectional Study to Compare Differences on Clinical and Laboratory Findings in Children with Seropositive and Seronegative Lupus

2021 ◽  
Author(s):  
Shima Salehi ◽  
Rozita Hosseini Shamsabadi ◽  
Hassan Otukesh ◽  
Reza Shiari ◽  
Monir Sharafi
Keyword(s):  
Autoimmune Disease ◽  
Disease Diagnosis ◽  
Cross Sectional Study ◽  
Rapid Identification ◽  
The Body ◽  
Musculoskeletal Symptoms ◽  
Laboratory Findings ◽  
Cross Sectional ◽  
Significant Difference ◽  
History Of

Abstract Background: Lupus is an inflammatory and autoimmune disease that involves various tissues and organs of the body. Identification of diagnostic elements to rapid identification of seronegative lupus cases is very important in order to prevent morbidity and progression of disease. This study aimed to compare clinical and laboratory findings of seropositive cases with seronegative lupus patients. Methods: This cross-sectional analytic study was performed on 43 children (17 seronegative and 26 seropositive) with lupus who were admitted to Ali Asghar Hospital during 2007-2017. Seropositive patients had anti-nuclear antibody (ANA) titration >1/80, while seronegative patients had ANA titration <1/80 (at the time of disease diagnosis). Clinical and laboratory findings were compared between two groups.Results: Serositis in patients with ANA- was significantly higher than ANA+ (41.17% vs. 23.07%; p = 0.042). ANA- group had higher autoimmune disease history than ANA+ group (42.85% vs. 15.0%; p = 0.041). The family history of the disease in the ANA- group was greater than ANA+ group (50% vs. 23.52%). The percentage of hypertensive patients in ANA- group was higher than ANA+ group (52.94% vs. 26.92%; p = 0.037). Neurologic symptoms in ANA+ and ANA- groups were 38.46% and 17.64%, respectively (p = 0.043). The frequency of patients with thrombocytopenia in ANA+ group was significantly greater than ANA- group (32% vs. 12.5%; p=0.041). There was no significant difference in other clinical and laboratory findings between two groups. Conclusion: Seronegative lupus patients had higher percentage of musculoskeletal symptoms, autoimmune disease history, familial history of disease, and hypertension, while neurological and thrombocytopenia symptoms were higher in seropositive patients compared to seronegative cases. Therefore, evaluation of these factors can be helpful to diagnosis of seronegative patients.

False-Positive D-Dimer Result in a Patient With Castleman Disease

2004 ◽  
Vol 128 (3) ◽  
pp. 328-331
Author(s):  
Kimberly Mugler ◽  
Jerry B. Lefkowitz
Keyword(s):  
Western Blot ◽  
Disseminated Intravascular Coagulation ◽  
False Positive ◽  
Degradation Products ◽  
False Negative ◽  
Castleman Disease ◽  
Laboratory Findings ◽  
Intravascular Coagulation ◽  
Immunodeficiency Virus ◽  
D Dimer

Abstract In suspected cases of disseminated intravascular coagulation, concurrent elevation of both fibrin(ogen) degradation products (FDPs) and D-dimer levels aids in confirming the diagnosis. This pattern of results reflects the action of plasmin proteolysis of cross-linked fibrin polymers as well as fibrinogen. We report the case of a patient with human immunodeficiency virus (HIV) and Castleman disease who presented with a high-positive D-dimer level and a negative FDP level in the course of a workup for disseminated intravascular coagulation. This finding suggested the possibility of either a false-positive D-dimer or a false-negative FDP level. To investigate the former, a Western blot was performed on the patient's serum to determine the presence of the D-dimer. No D-dimer band was visualized on the Western blot, confirming the false-positive nature of the D-dimer result. Insufficient quantity of patient serum, however, prevented further investigation into the etiology of this result. The false-positive D-dimer result is likely attributable to interference caused by the patient's Castleman disease–associated monoclonal gammopathy, a phenomenon that has been reported in other immunoassays. As the development of lymphoproliferative disorders is especially common within the HIV population, and hypergammaglobulinemia in Castleman disease is particularly common, clinicians should be aware of this phenomenon when the laboratory findings do not fit the clinical picture. Although it is rare, recognition of potential paraprotein interference in immunoassays will help avoid undertreatment or overtreatment of patients based on erroneous laboratory results.

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