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Case Reports in Hematology

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https://www.readbyqxmd.com/read/27891267/marginal-zone-lymphoma-complicated-by-protein-losing-enteropathy
#1
Nadine Stanek, Peter Bauerfeind, Guido Herzog, Henriette Heinrich, Matthias Sauter, Daniela Lenggenhager, Cäcilia Reiner, Markus G Manz, Jeroen S Goede, Benjamin Misselwitz
Protein losing enteropathy (PLE) refers to excessive intestinal protein loss, resulting in hypoalbuminemia. Underlying pathologies include conditions leading to either reduced intestinal barrier or lymphatic congestion. We describe the case of a patient with long-lasting diffuse abdominal problems and PLE. Repetitive endoscopies were normal with only minimal lymphangiectasia in biopsies. Further evaluations revealed an indolent marginal zone lymphoma with minor bone marrow infiltration. Monotherapy with rituximab decreased bone marrow infiltration of the lymphoma but did not relieve PLE...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27885347/fludarabine-treatment-of-patient-with-chronic-lymphocytic-leukemia-induces-a-digital-ischemia
#2
Utku Erdem Soyaltin, Deniz Yuce Yildirim, Mustafa Yildirim, Mehmet Can Ugur, Ferhat Ekinci, Cengiz Ceylan, Harun Akar
We report a 63-year-old man with a history of chronic lymphocytic leukemia (CLL) who presented with asymmetrical Raynaud's phenomenon of sudden onset which progressed to acral gangrene rapidly in a week. These symptoms began approximately one week after the fourth cycle of fludarabine and cyclophosphamide chemotherapy and were accompanied by pain, numbness, and cyanosis in the fingers of his right hand except the first finger. Fludarabine may play a role in acral vascular syndrome. The treatment with fludarabine in patients with evolving digital ischemia should be carried out with caution...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27882252/sj%C3%A3-gren-s-syndrome-complicated-by-myeloid-natural-killer-cell-precursor-acute-leukemia-case-report-and-review-of-the-literature
#3
Hao Feng, Jianlin Qiao, Ningning Ding, Wei Chen, Kunming Qi, Xiuying Pan, Jiang Cao, Kailin Xu
We report a case of Sjögren's syndrome (SS) complicated by myeloid/natural killer (NK) cell precursor acute leukemia (M/NKPAL). A 75-year-old woman with a previous SS history for 2 years was routinely treated. Peripheral blood progenitor cells were increased, and subsequent bone marrow cell morphology examination showed the presence of acute myeloid leukemia type M4. However, flow cytometry analysis revealed that CD7/CD56/CD33/CD34/HLA-DR/cCD3 were all positive and myeloperoxidase- (MPO-) specific staining, other T cells, NK cells, and myeloid markers were all negative...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27882251/bilateral-numb-chin-syndrome-as-the-initial-presentation-of-burkitt-s-lymphoma-leukemia-a-report-of-two-cases-and-review-of-the-literature
#4
Hussein Algahtani, Bader Shirah, Wafaa Bassuni, Reem Adas
Numb chin syndrome, also known as mental nerve neuropathy, is a rare sensory neuropathy characterized by paresthesia and hypoesthesia in the area supplied by the mental nerve and its branches. This syndrome may be the first symptom of underlying malignancy or the first sign of recurrence and metastasis in patients with preexisting cancer. In this article, we present two cases with bilateral numb chin syndrome as the first manifestation of Burkitt's lymphoma/leukemia and review the relevant literature. Numb chin syndrome should be considered as a warning sign and raise the suspicion for an underlying malignancy...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27872769/mantle-cell-hyperplasia-of-peripheral-lymph-nodes-as-initial-manifestation-of-sickle-cell-disease
#5
Ahmad Monabbati, Sadat Noori, Akbar Safaei, Mani Ramzi, Seyedsajjad Eghbali, Ali Adib
Sickle cell disease (SCD) is a well known hemoglobinopathy with usual manifestations including anemia, hyperbilirubinemia, and vasoocclusive complications. Despite presence of mild splenomegaly in early phase of the disease, lymphadenopathy is not an often finding of SCD. We introduce an undiagnosed case of SCD who presented in third decade of his life with multiple cervical lymphadenopathies and mild splenomegaly persistent for about five years. Histopathologic examination of the resected lymph nodes showed expansion of the mantle cell layers of secondary follicles as well as several monomorphic mantle cell nodules...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27872768/hip-replacement-surgery-in-14-year-old-girl-with-factor-v-deficiency-haemostatic-treatment-and-thromboprophylaxis
#6
María Eva Mingot-Castellano, Josefina Pérez-Núñez, Lourdes Baeza-Montañez
Factor V (FV) is a pivotal coagulation factor present in plasma and platelets. It plays an essential role in secondary haemostasis acting as a cofactor in the prothrombinase complex, catalysing the conversion of prothrombin to thrombin. There is little evidence on the management of mayor orthopaedic surgery in paediatric or adolescents subjects with this coagulopathy and almost no information about thromboprophylaxis in these situations. We report a case of a hip replacement in a 14-year-old girl with moderate FV deficiency (0...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27867671/retreatment-with-bendamustine-bortezomib-dexamethasone-in-a-patient-with-relapsed-refractory-multiple-myeloma
#7
Claudio Cerchione, Davide Nappi, Maria Di Perna, Irene Zacheo, Anna Emanuele Pareto, Marco Picardi, Lucio Catalano, Fabrizio Pane
The clinical management of relapsed/refractory multiple myeloma and the correct choice of the most suitable therapy in heavily pretreated and fragile patients are tough clinical issues for clinicians. In advanced phases of disease, the choice of available therapies becomes very poor, and the retreatment with previously adopted and effective therapy, although unpredictable, could be an effective option. In this report, we describe the clinical history of a patient, previously treated with 9 lines of therapy, refractory to bortezomib and IMIDs, for whom the retreatment with bendamustine resulted in a stable disease with good quality of life...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27867670/lymphoma-heterogeneity-three-different-histological-pictures-and-one-unique-clone
#8
Sara Alonso-Alvarez, Alba Redondo-Guijo, Óscar Blanco, Miguel Alcoceba, Ana Balanzategui, Juan C Caballero, Julio Dávila, Marcos González, María D Caballero, Alejandro Martín, Ramón García-Sanz
We report a patient who developed up to three different lymphomas with the same clonal IGH rearrangement. She was first diagnosed of splenic zone marginal lymphoma and relapsed for the first time with Hodgkin lymphoma histology and later with diffuse large B-cell lymphoma histology. Subsequent biopsies and analysis of clonally rearranged IGH genes helped to elucidate the clonal relationship between the three histologies and to confirm a common origin from the three tissue histologies. An integrated diagnosis should always be performed in order to achieve the most accurate diagnosis and be able to choose the best therapeutic options for our patients...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27843657/reactivation-of-pulmonary-tuberculosis-following-treatment-of-myelofibrosis-with-ruxolitinib
#9
Maheen Z Abidi, Javeria Haque, Parvathi Varma, Horatiu Olteanu, Guru Subramanian Guru Murthy, Binod Dhakal, Parameswaran Hari
Ruxolitinib is widely in use for treatment of myeloproliferative disorders. It causes inhibition of the Janus kinase (JAK) signal transducer and activation of transcription (STAT) pathway, which plays a key role in the underlying pathophysiology of myeloproliferative diseases. We describe a case of reactivation pulmonary tuberculosis in a retired physician while on treatment with ruxolitinib. We also review the literature on opportunistic infections following use of ruxolitinib. Our case highlights the importance of screening for latent tuberculosis in patients from highly endemic areas prior to start of therapy with ruxolitinib...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27840748/usefulness-of-low-dose-splenic-irradiation-prior-to-reduced-intensity-conditioning-regimen-for-hematopoietic-stem-cell-transplantation-in-elderly-patients-with-myelofibrosis
#10
Etsuko Matsubara, Jun Yamanouchi, Riko Kitazawa, Taichi Azuma, Hiroshi Fujiwara, Takaaki Hato, Masaki Yasukawa
The Janus kinase (JAK) 1 and 2 inhibitor, ruxolitinib, was recently approved in Japan and has been effective in many patients with myelofibrosis (MF). Although the inhibitor decreases splenomegaly and relieves MF-related symptoms, allogeneic hematopoietic cell transplantation (HCT) remains as the only curative therapy for MF. The presence of splenomegaly has been reported as a risk factor for graft failure, delayed engraftment, and poor survival. Here, we report two elderly MF patients with massive splenomegaly and a JAK2 V617F mutation...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27812394/balancing-therapy-with-thrombopoietin-receptor-agonists-and-splenectomy-in-refractory-immune-thrombocytopenic-purpura-a-case-of-postsplenectomy-thrombocytosis-requiring-plateletpheresis
#11
Jacquelyn Zimmerman, Kelly J Norsworthy, Robert Brodsky
Immune thrombocytopenic purpura (ITP) causes thrombocytopenia through the autoimmune destruction of platelets. Corticosteroids remain the first line of therapy, and traditionally splenectomy has been the second. While the availability of thrombopoietin receptor agonists (TPO-RAs) has expanded treatment options, there is little data for the ideal management of these agents in preparation for splenectomy. Thrombocytosis has been reported after splenectomy in patients treated with TPO-RA preoperatively, with one prior case requiring plateletpheresis for symptomatic thrombocytosis...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27807488/uveitis-and-myositis-as-immune-complications-in-chemorefractory-nk-t-cell-nasal-type-lymphoma-successfully-treated-with-allogeneic-stem-cell-transplant
#12
Maria José Gómez-Crespo, Aránzazu García-Raso, Jose Luis López-Lorenzo, Teresa Villaescusa, María Rodríguez-Pinilla, José Fortes, Cristina Serrano, Salma Machan, Pilar Llamas, Raúl Córdoba
NK/T-cell lymphomas are a group of clonal proliferations of NK- or, rarely, T-cell types and have peculiar clinicopathologic features. Most common site of involvement is the upper aerodigestive tract (nasal cavity, nasopharynx, paranasal sinuses, and palate). Association of autoimmune paraneoplastic disorders with NK/T-cell lymphomas is not well studied. Our patient was diagnosed with NK/T-cell lymphoma stage IV with skin involvement and treated frontline with CHOEP regimen. While he was under treatment, two immune complications presented: anterior uveitis of autoimmune origin refractory to steroids and myositis in lower limbs muscles...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27803823/massive-bleeding-as-the-first-clinical-manifestation-of-metastatic-prostate-cancer-due-to-disseminated-intravascular-coagulation-with-enhanced-fibrinolysis
#13
Mónica Palma Anselmo, Gustavo Nobre de Jesus, João Madeira Lopes, Rui M M Victorino, João Meneses Santos
Disseminated intravascular coagulation (DIC) is the most frequent coagulation disorder associated with metastatic prostate adenocarcinoma. However, DIC with enhanced fibrinolysis as an initial presentation of prostate cancer is extremely rare. The appropriate treatment to control bleeding in these situations is challenging, controversial, and based on isolated case reports in the literature. A 66-year-old male presented at the emergency department with acute severe spontaneous ecchymoses localized to the limbs, laterocervical hematoma, and hemothorax...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27803822/pancytopenia-in-a-patient-with-rendu-osler-weber-syndrome-and-uncommon-vascular-abnormalities
#14
Nicolò Binello, Antonio Gasbarrini, Eleonora Gaetani
Rendu-Osler-Weber syndrome, or hereditary hemorrhagic teleangiectasia (HHT), is a rare autosomal dominant vascular disorder, characterized by multiple mucocutaneous teleangiectases with recurrent nasal and gastrointestinal bleedings and/or solid-organ arteriovenous shunts. We describe the first case to our knowledge of pancytopenia in a 53-year-old patient, with a known history of HHT and recurrent nasal and gastrointestinal bleedings, who was found to have a major splenic artery aneurysm and other uncommon vascular abnormalities...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27803821/hemophagocytic-lymphohistiocytosis-in-a-patient-with-classical-hodgkin-lymphoma
#15
G Hyun, K J Robbins, N Wilgus, L Grosso, S D Goyal
Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome that can be associated with inherited genetic mutations, malignancy, autoimmune disorders, and viral infections. Though the pathogenesis is not fully known, HLH is understood to be a reactive process in the setting of uncontrolled activation of macrophages, CD8+ cytotoxic lymphocytes, and other immune cells. Hallmark clinicopathological features of HLH include fevers, cytopenias, hepatosplenomegaly, and hemophagocytosis in the bone marrow...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27800196/chronic-disseminated-candidiasis-complicated-by-immune-reconstitution-inflammatory-syndrome-in-child-with-acute-lymphoblastic-leukemia
#16
Olga Zając-Spychała, Bogna Ukielska, Katarzyna Jończyk-Potoczna, Benigna Konatkowska, Jacek Wachowiak
Hepatosplenic candidiasis also known as chronic disseminated candidiasis is a rare manifestation of invasive fungal infection typically observed in patients with acute leukemia in prolonged, deep neutropenia. Immune reconstitution inflammatory syndrome (IRIS) is an inflammatory disorder triggered by rapid resolution of neutropenia. Diagnosis and treatment of IRIS are still challenging due to a variety of clinical symptoms, lack of certain diagnostic criteria, and no standards of treatment. The diagnosis of IRIS is even more difficult in patients with hematological malignancies complicated by "probable" invasive fungal infection, when fungal pathogen is still uncertain...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27777803/a-case-of-de-novo-cd5-disseminated-intravascular-large-b-cell-lymphoma-presenting-as-multiorgan-failure
#17
Daulath Singh, Devika Kapuria, Suparna Nanua, Rakesh Gaur
Intravascular large B-cell lymphoma is an extremely rare extranodal lymphoma that proliferates in the lumen of the blood vessels while sparing the organ parenchyma. It usually presents with CNS and skin involvement. A 65-year-old Caucasian female presented with fevers and chills of 3-4 months' duration. Bone marrow biopsy done 3 months prior showed no significant myelodysplasia or lymphoid aggregates. The patient later died due to multiorgan failure. A bone marrow biopsy showed 20-30% CD5+ B cells consistent with infiltrative large B-cell lymphoma...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27777802/tafro-syndrome-associated-with-ebv-and-successful-triple-therapy-treatment-case-report-and-review-of-the-literature
#18
Malorie Simons, Emmanuel Apor, James N Butera, Diana O Treaba
TAFRO syndrome is a rare constellation of symptoms: thrombocytopenia, anasarca, reticulin fibrosis of the bone marrow, renal dysfunction, and organomegaly. Its pathogenesis involves an excessive and inappropriate cytokine storm, most notably from IL-6, causing multiorgan failure; however, its etiology is undetermined. Starting in 2012, TAFRO syndrome was first identified in Japan as an atypical variant of Castleman's disease. Previous reports include various different treatment protocols with inconsistent survival outcomes...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27774324/cd7-positive-diffuse-large-b-cell-lymphoma-arising-in-a-background-of-follicular-lymphoma-a-case-report-and-review-of-the-literature
#19
Elham Vali Betts, Hooman H Rashidi
Diffuse large B-cell lymphoma (DLBCL) is a neoplasm of large B-lymphocytes with a diffuse growth pattern. The neoplastic cells express B-cell markers such as CD20 and PAX-5 and there may be coexpression of BCL-2, BCL-6, CD10, and MUM-1. With the exception of CD5, other T-cell markers are not commonly expressed in this neoplasm. Here, we describe the first reported case of a DLBCL with abnormal expression CD7 arising in a background of follicular lymphoma in an 81-year-old male who presented with a nontender left axillary mass...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27752371/myeloproliferative-neoplasm-or-reactive-process-a-rare-case-of-acute-myeloid-leukemia-and-transient-posttreatment-megakaryocytic-hyperplasia-with-jak-2-mutation
#20
Steven Wang, Jie Yan, Guangde Zhou, Rebecca Heintzelman, J Steve Hou
Myeloproliferative neoplasms (MPNs) are hematopoietic malignancies characterized by unchecked proliferation of differentiated myeloid cells. The most common BCR-ABL1-negative MPNs are polycythemia vera, essential thrombocythemia, and primary myelofibrosis. The discovery of JAK2 V617F mutation has improved our understanding of the molecular basis of MPN. The high frequency of JAK2 mutation in MPN makes JAK2 mutation testing an essential diagnostic tool and potential therapeutic target for MPN. Here, we present a rare case of a 34-year-old patient who was initially diagnosed with acute myeloid leukemia (AML) with mutated NPM1...
2016: Case Reports in Hematology
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