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https://www.readbyqxmd.com/read/29780126/a-case-of-intractable-hemophagocytic-syndrome-associated-with-systemic-lupus-erythematosus-resistant-to-corticosteroids-and-intravenous-cyclophosphamide-that-was-successfully-treated-with-cyclosporine-a
#1
Hirofumi Toko, Hiroto Tsuboi, Naoto Umeda, Fumika Honda, Ayako Ohyama, Hidenori Takahashi, Saori Abe, Masahiro Yokosawa, Hiromitsu Asashima, Shinya Hagiwara, Tomoya Hirota, Yuya Kondo, Isao Matsumoto, Takayuki Sumida
Hemophagocytic syndrome (HPS) associated with systemic lupus erythematosus (SLE), dubbed acute lupus hemophagocytic syndrome (ALHS), is an intractable complication of SLE. A 24-year-old man who had been diagnosed with SLE three months previously, presented with fever, rash, hallucination, and pancytopenia accompanied with hyperferritinemia and bone marrow hemophagocytosis. He was diagnosed with ALHS and neuropsychiatric (NP)-SLE. Although 4 courses of methylprednisolone pulse therapy and 1 course of intravenous cyclophosphamide (IVCY) improved his NP-SLE, his ALHS did not respond...
May 18, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29762163/transcriptional-gene-silencing-limits-cxcr4-associated-depletion-of-bone-marrow-cd34-cells-in-hiv-1-infection
#2
Tetsuo Tsukamoto
OBJECTIVES: Hematological abnormalities that include changes in bone marrow, such as in anemia and pancytopenia, are common among human immunodeficiency virus (HIV)-infected patients, particularly in the advanced stage of disease. Such abnormalities may be caused by a reduced bone marrow function for hematopoiesis. This aim of this study was to determine whether transcriptional gene silence can help to preserve the hosts' hematopoietic potential in addition to peripheral CD4 T cells against CCR5-tropic HIV infection...
May 11, 2018: AIDS
https://www.readbyqxmd.com/read/29754142/cholestatic-jaundice-a-unique-presentation-leading-to-the-diagnosis-of-hlh-with-hodgkin-lymphoma-hiv-and-ebv
#3
Hira Shaikh, Soorih Shaikh, Amir Kamran, Prerna Mewawalla
Haemophagocytic lymphohistiocytosis (HLH) is a syndrome of dysregulated immune activity with macrophage activation that can manifest as pancytopenia, coagulopathy and other laboratory abnormalities, usually progressing to multiorgan failure and death. This report documents the rarely reported association between HLH and Hodgkin's lymphoma (HL) with simultaneous HIV and Epstein-Barr virus (EBV) and complete resolution with chemotherapy. The patient initially presented with cholestatic jaundice. He was then found to have HL associated with HLH with coexistent HIV and EBV viraemia...
May 12, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29750741/hemophagocytic-lymphohystiocytosis-associated-with-type-ia-glycogen-storage-disease
#4
Yeter Düzenli Kar, Zeynep C Özdemir, Eylem Kiral, Gonca Kiliç Yildirim, Ener Ç Dinleyici, Özcan Bör
BACKGROUND: Hemophagocytic lymphohystiocytosis (HLH) is characterized by fever, splenomegaly, pancytopenia, and elevated levels of triglycerides and ferritin. These signs and symptoms are common to other metabolic diseases. OBSERVATION: A 5-month-old female infant, who presented with fever, respiratory distress, massive hepatomegaly, and bicytopenia, was diagnosed as having HLH and chemotherapy was initiated. The patient was negative for familial HLH gene mutations...
May 10, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29749364/cytopenia-in-adult-brucellosis-patients
#5
Safak Kaya, Nazif Elaldi, Ozcan Deveci, Ahmet Emre Eskazan, Muhammed Bekcibasi, Salih Hosoglu
Background & objectives: Brucellosis can lead to haematological abnormalities including cytopenia confusing with haematological malignancies. The aim of this study was to compare the main characteristics of brucellosis patients without cytopenia (Group 1) and with cytopenia (Group 2). Methods: This five-year period study which was performed in two referral hospitals in Turkey, included all adult brucellosis patients. Abnormally, low counts of leucocyte or haemoglobin or platelets in a patient were considered as cytopenia...
January 2018: Indian Journal of Medical Research
https://www.readbyqxmd.com/read/29749240/why-is-it-necessary-to-examine-retina-when-the-patient-suffers-from-aplastic-anemia
#6
D Tomcikova, A Gerinec, B Busanyova, M Gresikova, S Biskup, K Hortnagel
PURPOSES: To inform about a case of Revesz syndrome (RS) with initial ophthalmological symptomatology of severe proliferative vitreoretinopathy of the left eye (LE). After the aplastic anemia had developed, RS was established. The exudative retinopathy was successfully treated with photocoagulation on the right eye (RE). BACKGROUND: RS is characterized by fatal bone marrow failure, exudative retinopathy, neuroradiographic abnormalities, neurodevelopmental delay and skin abnormalities...
2018: Bratislavské Lekárske Listy
https://www.readbyqxmd.com/read/29746437/hematologic-findings-of-inherited-metabolic-disease-they-are-more-than-expected
#7
Ertan Sal, Idil Yenicesu, Ilyas Okur, Zuhre Kaya, Fatih S Ezgu, Ulker Kocak, Leyla Tumer, Turkiz Gursel, Alev Hasanoglu
Inherited metabolic diseases are pathologic conditions that generally develop as a result of impairment of the production or breakdown of protein, carbohydrate, and fatty acids. Early determination of hematological findings has a positive effect on the prognosis of metabolic diseases. Three hundred eighteen patients who were being followed-up within the previous 6 months at Department of Pediatric Nutrition and Metabolism, Gazi University, Turkey, were included in the study. The hematological findings were classified under 7 main groups: anemia of chronic disease, iron deficiency anemia, vitamin B12 deficiency anemia, hemophagocytosis, leukocytosis, and thrombocytosis...
May 9, 2018: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29743401/-hypoplastic-acute-promyelocytic-leukemia-with-continuous-hypocellular-bone-marrow-after-remission
#8
Toshiki Nakamura, Junya Makiyama, Ayumi Matsuura, Hirokazu Kurohama, Hideaki Kitanosono, Masahiro Ito, Shinichiro Yoshida, Yasushi Miyazaki
An 87-year old female presented with unsteady gait and occasional subcutaneous hematomas. Blood examination findings revealed pancytopenia and mild coagulopathy. Both the histopathological evaluation of bone marrow smears and bone marrow biopsy revealed a hypocellular bone marrow. However, APL cells were observed and PML-RARA fusion gene was detected. On the basis of these findings, the patient was diagnosed with hypoplastic acute promyelocytic leukemia. She received ATRA treatment and achieved complete remission (CR) 29 days from the commencement of therapy...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29742693/a-successful-treatment-of-severe-systemic-lupus-erythematosus-caused-by-occult-pulmonary-infection-associated-with-hemophagocytic-syndrome-a-case-report
#9
Weihong Shi, Mingyang Duan, Ligang Jie, Weifeng Sun
RATIONALE: A 27-year-old woman with a history of systemic lupus erythaematosus (SLE) developed hemophagocytic syndrome (HPS) secondary due to an unrecognized infection that led to severe SLE with a prolonged recovery. PATIENT CONCERNS: The patient showed a high spiking fever and myalgia. Laboratory data revealed pancytopenia and immunological abnormalities. Pulse methylprednisone plus intravenous immunoglobulin (IVIG) failed to improve the clinical symptoms and laboratory data...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29742076/update-on-hairy-cell-leukemia
#10
Robert J Kreitman, Evgeny Arons
Hairy cell leukemia (HCL) is a chronic B-cell malignancy with multiple treatment options, including several that are investigational. Patients present with pancytopenia and splenomegaly, owing to the infiltration of leukemic cells expressing CD22, CD25, CD20, CD103, tartrate-resistant acid phosphatase (TRAP), annexin A1 (ANXA1), and the BRAF V600E mutation. A variant lacking CD25, ANXA1, TRAP, and the BRAF V600E mutation, called HCLv, is more aggressive and is classified as a separate disease. A molecularly defined variant expressing unmutated immunoglobulin heavy variable 4-34 (IGHV4-34) is also aggressive, lacks the BRAF V600E mutation, and has a phenotype of HCL or HCLv...
March 2018: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/29736106/a-novel-mitochondrial-dna-deletion-in-patient-with-pearson-syndrome
#11
Rame Khasawneh, Hala Alsokhni, Bayan Alzghoul, Asim Momani, Nazih Abualsheikh, Nazmi Kamal, Mousa Qatawneh
Introduction: Arteriovenous Pearson syndrome is a very rare multisystemic mitochondrial disease characterized by sideroblastic anemia and exocrine pancreatic insufficiency. It is usually fatal in infancy. Case report: We reported a four-month-old infant presented with fever and pancytopenia. Bone marrow examination showed hypoplastic changes and sideroblastic features. Molecular Study showed a novel hetroplasmic mitochondrial deletions (m. 10760 -m. 15889+) in multiple genes (ND4,ND5,ND6, CYTB)...
April 2018: Medical Archives
https://www.readbyqxmd.com/read/29722478/successful-reduced-intensity-conditioning-hematopoietic-stem-cell-transplantation-for-paroxysmal-nocturnal-hemoglobinuria-with-aplastic-anemia-in-two-children
#12
Jeffrey R Andolina, Ariel L Reinish, Razia Akhtar, Suzie Noronha, Jessica C Shand, Angela Girvin, David N Korones, Lauren B Bruckner, Craig A Mullen, Kevin J Curran, Farid Boulad
Paroxysmal nocturnal hemoglobinuria (PNH) is an extremely rare cause of bone marrow failure in children. We report two children who presented with pancytopenia, and were diagnosed with PNH with severe aplastic anemia. Both children underwent upfront, successful hematopoietic stem cell transplantation with reduced-intensity conditioning. One patient had a syngeneic donor, and one patient had a 10/10 matched unrelated donor. Neither patient developed graft versus host disease, infections, or recurrent PNH. Reduced-intensity conditioning hematopoietic stem cell transplantation is a reasonable therapy for PNH with marrow failure in children...
May 3, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29712885/leishmania-in-a-patient-with-small-lymphocytic-lymphoma-chronic-lymphocytic-leukemia
#13
Gregory Nicolas, Elliott Koury, Dani Osman, Christian Salibi, Lea Nehme, Stephanie Mitri, Julien-Sami Atef El Sayegh, Linda Rached, George Khoury
BACKGROUND Leishmaniasis is a parasitic infection spread by the bite of infected sand flies that are usually present in the Middle East, Africa, and some parts of Asia and Europe. Leishmaniasis manifests in 3 different forms: Visceral (also known as Kala Azar), which is the most serious type; cutaneous, which is the most common type; and mucocutaneous. The symptoms of this infection range from a silent infection to fever, enlargement of the liver and spleen, weight loss, and pancytopenia. CASE REPORT In this case report, we discuss a 73-year-old man known to have chronic lymphocytic leukemia (CLL), presenting with unremitting fever and who to our surprise was found to have Kala Azar...
May 1, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29703015/a-case-report-of-a-very-late-response-to-5-azacytidine-in-a-patient-with-lower-risk-myelodysplastic-syndrome-time-to-update-treatment-guidelines-for-lower-risk-patients
#14
Nikolaos Spetsieris, Panagiotis Diamantopoulos, Konstantinos Zervakis, Nefeli Giannakopoulou, Niki Rougala, Georgios Garefalakis, Vasiliki Skarlatou, Nora-Athina Viniou
RATIONALE: The hypomethylating agent 5-azacytidine has been approved in Europe for patients with intermediate 2 and high (i.e., higher) risk myelodysplastic syndrome according to the International Prognostic Scoring System (IPSS). A total of 91% of all first responses in higher risk patients occur within 6 cycles of treatment; however, data regarding the time to first response in clinical trials with lower risk patients are not available. PATIENT CONCERNS: Our case describes the late response of a lower risk (intermediate 1 according to the IPSS and intermediate according to the IPSS-R) patient to 5-azacytidine treatment...
April 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29686571/-bartonella-endocarditis-with-glomerulonephritis-in-a-patient-with-complete-transposition-of-the-great-arteries
#15
Helen Hashemi, Tiana R Endicott-Yazdani, Christopher Oguayo, David M Harmon, Tuan Tran, Ginger Tsai-Nguyen, Raul Benavides, Cedric W Spak, Hoang-Lan Nguyen
We describe a patient with history of dextro-transposition of the great vessels, ventricular septal defect, and pulmonary valve replacement who presented with fatigue, prolonged fever, and leg edema. He was found to have kidney injury, pancytopenia, and liver congestion. Echocardiogram revealed thickened leaflets with prolapsing vegetation on the pulmonary valve. Given the negative blood cultures, high Bartonella henselae immunogobulin G titer (≥1:1024) and positive immunoglobulin M titer (≥1:20), he was diagnosed with Bartonella endocarditis complicated with glomerulonephritis...
January 2018: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/29685167/neutropenia-caused-by-hairy-cell-leukemia-in-a-patient-with-myelofibrosis-secondary-to-polycythemia-vera-a-case-report
#16
Andreas Hanssønn Habberstad, Hoa Thi Tuyet Tran, Ulla Randen, Signe Spetalen, Ingunn Dybedal, Geir E Tjønnfjord, Anders Erik Astrup Dahm
BACKGROUND: Polycythemia vera is a myeloproliferative disease that sometimes evolves to myelofibrosis, causing splenomegaly and neutropenia. In this case report, we describe a patient with polycythemia vera and unexplained neutropenia who later turned out to also have hairy cell leukemia. CASE PRESENTATION: A middle-aged Caucasian man with polycythemia vera presented to our hospital with chronic mouth ulcers. Later he developed leukopenia and pancytopenia. Bone marrow biopsies showed fibrosis...
April 24, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29682050/spastic-quadriparesis-and-communicating-hydrocephalus-as-late-sequel-of-rosai-dorfman-disease-a-case-report-and-review-of-literature
#17
Ankur Kapoor, Pravin Salunke, Chirag K Ahuja, Debjyoti Chatterjee
Rosai-Dorfman disease (RDD) predominantly affects cervical lymph nodes and presents with fever and pancytopenia. Central nervous system involvement though uncommon is often reported. Hydrocephalus and paraparesis as a consequence of RDD is an extremely rare entity. We present a 58-year-old male, diagnosed and treated for RDD with cervical lymphadenopathy, who now presented with spastic paraparesis and on evaluation was found to have communicating hydrocephalus that resolved after ventriculoperitoneal shunt surgery...
April 2018: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/29673488/corrigendum-to-cd41-immune-staining-of-micromegakaryocytes-improves-the-diagnosis-of-myelodysplastic-syndrome-and-differentiation-from-pancytopenia-leukemia-research-2018-66-15-19
#18
Jinjin Huang, Zhiqiong Wang, Lifang Huang, Miao Zheng
No abstract text is available yet for this article.
May 2018: Leukemia Research
https://www.readbyqxmd.com/read/29671215/a-patient-presenting-with-isolated-hematuria-and-renal-dysfunction-as-rare-manifestation-of-cryoglobulinemic-glomerulonephritis-in-the-course-of-autoimmune-diseases-including-sj%C3%A3-gren-s-syndrome
#19
Masaki Yamanaka, Yoshihide Fujigaki, Hajime Kono, Michito Nagura, Shigeyuki Arai, Yoshifuru Tamura, Tatsuru Ota, Shigeru Shibata, Fukuo Kondo, Yutaka Yamaguchi, Shunya Uchida
Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren's syndrome are most frequent condition among non-hepatitis C virus-related condition. Its typical renal manifestation shows high amount of proteinuria with microscopic hematuria and renal insufficiency. We describe a case of 72-year-old woman with Hashimoto disease, autoimmune hepatitis, Sjögren's syndrome, and immune-related pancytopenia complicated by cryoglobulinemic glomerulonephritis...
April 18, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29670776/-kit-d816v-positive-acute-mast-cell-leukemia-associated-with-normal-karyotype-acute-myeloid-leukemia
#20
Marta Lopes, Maria Dos Anjos Teixeira, Cláudia Casais, Vanessa Mesquita, Patrícia Seabra, Renata Cabral, José Palla-García, Catarina Lau, João Rodrigues, Maria Jara-Acevedo, Inês Freitas, Jose Ramón Vizcaíno, Jorge Coutinho, Luis Escribano, Alberto Orfao, Margarida Lima
Introduction: Mast cell (MC) leukemia (MCL) is extremely rare. We present a case of MCL diagnosed concomitantly with acute myeloblastic leukemia (AML). Case Report: A 41-year-old woman presented with asthenia, anorexia, fever, epigastralgia, and diarrhea. She had a maculopapular skin rash, hepatosplenomegaly, retroperitoneal adenopathies, pancytopenia, 6% blast cells (BC) and 20% MC in the peripheral blood, elevated lactate dehydrogenase, cholestasis, hypoalbuminemia, hypogammaglobulinemia, and increased serum tryptase (184  μ g/L)...
2018: Case Reports in Hematology
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