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Case Reports in Hematology

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https://www.readbyqxmd.com/read/28428897/acute-myeloid-leukemia-with-basophilic-differentiation-transformed-from-myelodysplastic-syndrome
#1
Yasuhiro Tanaka, Atsushi Tanaka, Akiko Hashimoto, Kumiko Hayashi, Isaku Shinzato
Myelodysplastic syndrome (MDS) terminally transforms to acute myeloid leukemia (AML) or bone marrow failure syndrome, but acute myeloid leukemia with basophilic differentiation has been rarely reported. An 81-year-old man was referred to our department for further examination of intermittent fever and normocytic anemia during immunosuppressive treatment. Chromosomal analysis showed additional abnormalities involving chromosome 7. He was diagnosed as having MDS. At the time of diagnosis, basophils had not proliferated in the bone marrow...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28424750/celiac-disease-presenting-with-immune-thrombocytopenic-purpura
#2
Hakan Sarbay, Billur Cosan Sarbay, Mehmet Akın, Halil Kocamaz, Mahya Sultan Tosun
Celiac disease (CD) is an immunological disorder. Clinical manifestations occur as a result of intestinal mucosa damage and malabsorption. CD is also associated with extraintestinal manifestations and autoimmune disorders. The coexistence of CD and autoimmune diseases has been described before. In this article, a patient with CD presenting with thrombocytopenia is discussed.
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28421151/extramedullary-relapse-in-a-cml-patient-after-allogeneic-stem-cell-transplantation
#3
Asu Fergun Yilmaz, Nur Soyer, Nazan Ozsan, Seckin Cagirgan, Ajda Gunes, Melda Comert, Fahri Sahin, Guray Saydam, Nur Selvi Gunel, Filiz Vural
Myeloid or granulocytic sarcoma (GS) is a tumoral lesion consisting of immature granulocytic cells. It is a rare entity during the course of CML patients especially after allogeneic stem cell transplantation (SCT). Relapse without bone marrow involvement is much rarer. We report a case of CML patient who relapsed with isolated granulocytic sarcoma after allogeneic SCT during cytogenetic and molecular remission. 28-year-old male was diagnosed as CML and allogeneic SCT was performed because of refractory disease to tyrosine kinase inhibitors...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28409040/a-case-of-blastic-plasmacytoid-dendritic-cell-neoplasm-extensively-studied-by-flow-cytometry-and-immunohistochemistry
#4
Martina Pennisi, Clara Cesana, Micol Giulia Cittone, Laura Bandiera, Barbara Scarpati, Valentina Mancini, Silvia Soriani, Silvio Veronese, Mauro Truini, Silvano Rossini, Roberto Cairoli
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive clinical course and poor prognosis. Diagnosis is based on detection of CD4(+) CD56(+), CD123(high), TCL-1(+), and blood dendritic cell antigen-2/CD303(+) blasts, together with the absence of lineage specific antigens on tumour cells. In this report we present a case of BPDCN presenting with extramedullary and bone marrow involvement, extensively studied by flow cytometry and immunohistochemistry, who achieved complete remission after acute lymphoblastic leukemia like chemotherapy and allogeneic hematopoietic stem cell transplantation...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28367342/remission-of-severe-relapsed-and-refractory-ttp-after-multiple-cycles-of-bortezomib
#5
Manu R Pandey, Pankit Vachhani, Evelena P Ontiveros
Acquired thrombotic thrombocytopenic purpura (TTP) is characterized by autoantibodies against a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Uncleaved von Willebrand factor (VWF) multimers accumulate and bind to platelets which causes spontaneous microthrombi ultimately causing microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemia. Plasma exchange (PEX) with or without steroids constitutes standard first-line therapy with rituximab typically reserved for refractory cases...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28367341/hereditary-xerocytosis-due-to-mutations-in-piezo1-gene-associated-with-heterozygous-pyruvate-kinase-deficiency-and-beta-thalassemia-trait-in-two-unrelated-families
#6
Elisa Fermo, Cristina Vercellati, Anna Paola Marcello, Anna Zaninoni, Richard van Wijk, Nadia Mirra, Cristina Curcio, Agostino Cortelezzi, Alberto Zanella, Wilma Barcellini, Paola Bianchi
Hereditary xerocytosis (HX) is a rare disorder caused by defects of RBC permeability, associated with haemolytic anaemia of variable degree and iron overload. It is sometimes misdiagnosed as hereditary spherocytosis or other congenital haemolytic anaemia. Splenectomy is contraindicated due to increased risk of thromboembolic complications. We report the clinical, haematological, and molecular characteristics of four patients from two unrelated Italian families affected by HX, associated with beta-thalassemia trait and heterozygous pyruvate kinase deficiency, respectively...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28326208/a-child-as-a-donor-for-hematopoietic-stem-cell-transplantation-bioethical-justification-a-case-study-on-sickle-cell-disease
#7
Andrea Z Pereira, Ricardo Hellman, Nelson Hamerschlak, Andrea Kondo, Polianna Mara Rodrigues de Souza, Wilson Leite Pedreira, Luiz Fernando Alves Lima Mantovani, Eduardo Juan Troster, Henrique Grunspun, Marco Aurélio Scarpinella Bueno
Hematopoietic stem cell transplantation (HSCT) is an important treatment option for children with severe and refractory sickle cell disease (SCD) with debilitating clinical complications. HSCT with cells from the bone marrow of a HLA-identical sibling used in SCD has a low mortality risk, high cure rate, and high event-free survival rate after a median follow-up of 5-6 years. However, matched donors are found in only about 20% of the patients. A boy aged 8 years with SCD had a sister, <2 years old, a fully compatible donor...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28326207/dasatinib-and-prednisolone-induction-therapy-for-a-case-of-philadelphia-chromosome-positive-acute-lymphoblastic-leukemia-with-dilated-cardiomyopathy-accompanied-by-life-threatening-ventricular-tachycardia
#8
Mitsutaka Nishimoto, Hirohisa Nakamae, Kana Matsumoto, Kunihiko Morita, Yuki Koga, Dai Momose, Masayuki Hino
A 56-year-old man being treated for dilated cardiomyopathy presented with epigastralgia. He was diagnosed with ventricular tachycardia and Philadelphia chromosome-positive acute lymphoblastic leukemia. After treating incessant ventricular tachycardia, we commenced induction therapy for leukemia with dasatinib and prednisolone to minimize toxicity towards cardiomyocytes and the cardiac conduction system. Although dasatinib was temporarily withheld because of a recurrence of ventricular tachycardia, we rechallenged dasatinib while using bisoprolol and amiodarone and achieved a complete hematological response three weeks later...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28321349/uncovering-clinical-features-of-de-novo-philadelphia-positive-myelodysplasia
#9
Aristides Armas, Chen Chen, Martha Mims, Gustavo Rivero
Myelodysplastic syndrome (MDS) is cytogenetically heterogeneous and retains variable risk for acute myeloid leukemia transformation. Though not yet fully understood, there is an association between genetic abnormalities and defects in gene expression. The functional role for infrequent cytogenetic alteration remains unclear. An uncommon chromosomic abnormality is the presence of the Philadelphia (Ph) chromosome. Here, we report a patient with Ph+ MDS treated with low dose Dasatinib who achieved hematologic response for 7 months...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28321348/spontaneous-remission-of-an-untreated-myc-and-bcl2-coexpressing-high-grade-b-cell-lymphoma-a-case-report-and-literature-review
#10
D Alan Potts, Jonathan R Fromm, Ajay K Gopal, Ryan D Cassaday
Non-Hodgkin lymphomas (NHL) are a heterogeneous group of hematologic malignancies typically treated with multiagent chemotherapy. Rarely, spontaneous remissions can be observed, particularly in more indolent subtypes. The prognosis of aggressive NHL can be predicted using clinical and histopathologic factors. In aggressive B-cell NHL, the importance of MYC and BCL2 proto-oncogene coexpression (as assessed by immunohistochemistry) and high-grade histologic features are particularly noteworthy. We report a unique case of spontaneous remission in a patient with an aggressive B-cell NHL which harbored high-risk histopathologic features, including MYC protein expression at 70-80%, BCL2 protein expression, and morphologic features suggestive of high-grade B-cell lymphoma, NOS (formerly B-cell lymphoma unclassifiable with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma [BCLU])...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28316847/malignant-lymphoma-in-the-psoas-major-muscle
#11
Nobuhiro Akuzawa, Takashi Hatori, Aya Takase, Jun Aoki, Shinji Sakurai, Masahiko Kurabayashi
An 84-year-old Japanese man taking warfarin to prevent cerebral infarction secondary to atrial fibrillation was admitted to our hospital for evaluation of a painless right back mass. Magnetic resonance imaging (MRI) showed an oval-shaped mass in the right psoas major muscle. The mass showed high intensity on T1-, T2-, and diffusion-weighted imaging and mimicked an acute-phase hematoma. However, it showed no chronological changes typical of a hematoma, and MRI revealed enlargement of the mass 1 week after admission...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28316846/donor-derived-smoldering-multiple-myeloma-following-a-hematopoietic-cell-transplantation-for-aml
#12
Bita Fakhri, Mark Fiala, Michael Slade, Peter Westervelt, Armin Ghobadi
Posttransplant Lymphoproliferative Disorder (PTLD) is one of the most common malignancies complicating solid organ transplantation. In contrast, PTLD accounts for a minority of secondary cancers following allogeneic hematopoietic cell transplantation (HCT). Here we report on a 61-year-old woman who received an ABO-mismatched, HLA-matched unrelated donor hematopoietic cell transplantation from a presumably healthy donor for a diagnosis of acute myeloid leukemia (AML). Eighteen months following her transplant, she developed a monoclonal gammopathy...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28286680/utility-of-myd88-in-the-differential-diagnosis-and-choice-of-second-line-therapy-in-a-case-of-nonsecretory-lymphoplasmacytic-lymphoma-versus-free-light-chain-waldenstrom-s-macroglobulinemia
#13
D Kazmierski, M L Palomba, C Barsigian
The MYD88 L265P somatic variant (MYD88) has a high prevalence in Waldenstrom's Macroglobulinemia (WM), a form of lymphoplasmacytic lymphoma (LPL) associated with monoclonal IgM. Although the role of MYD88 in WM was initially reported in 2012, it was not until 2016 that MYD88 testing was included in the National Cancer Care Network (NCCN) Guidelines. We present a case illustrating the utility of MYD88 status in distinguishing atypical forms of WM from marginal zone lymphoma (MZL) and in selecting second-line therapy with ibrutinib...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28280640/hhv8-ebv-coinfection-lymphoproliferative-disorder-rare-entity-with-a-favorable-outcome
#14
Dhouha Bacha, Beya Chelly, Houda Kilani, Lamia Charfi, Amel Douggaz, Samia Chatti, Emna Chelbi
HHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD) is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent 78-year-old woman. The diagnosis was made in an incidentally discovered lymphadenopathy. Histological examination showed a nodular lymphoid proliferation centered by aggregates of atypical plasmablastic cells admixed with small lymphoid cells...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28270940/synchronous-occurrence-of-chronic-myeloid-leukemia-and-mantle-cell-lymphoma
#15
Prajwol Pathak, Ying Li, Brian Allen Gray, William Stratford May, Merry Jennifer Markham
Chronic myeloid leukemia (CML) and mantle cell lymphoma (MCL) are hematologic malignancies that originate from different oligopotent progenitor stem cells, namely, common myeloid and lymphoid progenitor cells, respectively. Although blastic transformation of CML can occur in the lymphoid lineage and CML has been related to non-Hodgkin lymphoma on transformation, to our knowledge, de novo and synchronous occurrence of CML and MCL has not been reported. Herein, we report the first case of synchronous CML and MCL in an otherwise healthy 38-year-old man...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28261509/spontaneous-heparin-induced-thrombocytopenia-and-venous-thromboembolism-following-total-knee-arthroplasty
#16
Kevin Baker, Ming Y Lim
A 72-year-old Caucasian woman was admitted for an elective left total knee arthroplasty. Her surgery was uncomplicated and she was discharged to a rehabilitation facility. Twelve days later, she developed acute shortness of breath followed by a syncopal episode. She was hypoxic and cyanotic, requiring hospitalization and intubation, and was subsequently diagnosed with bilateral submassive pulmonary emboli and bilateral lower extremity deep vein thrombosis. She was started on unfractionated heparin infusion...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28255478/myelodysplastic-syndrome-clinically-presenting-with-the-classic-ttp-pentad
#17
Santiago Fabián Moscoso Martínez, Evelyn Carolina Polanco Jácome, Elizabeth Guevara, Vijay Mattoo
The clinical presentation of myelodysplastic syndrome (MDS) is not specific. Many patients can be asymptomatic and can be detected only due to an abnormal complete blood cell count (CBC) on routine exam or for other reasons while others can be symptomatic as a consequence of underlying cytopenias. Thrombotic thrombocytopenic purpura (TTP) usually is suspected under the evidence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia and because it is a life-threatening condition (medical emergency) immediate initiation of plasmapheresis could be life-saving...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28197348/angioimmunoblastic-t-cell-lymphoma-a-questionable-association-with-follicular-dendritic-cell-sarcoma
#18
Daniel Benharroch, Miriam Zekzer, Karen Nalbandyan
An elderly woman presented with generalized lymphadenopathy, several systemic symptoms, and splenomegaly. An inguinal lymph node excision revealed a compound picture. One aspect of the lymph node morphology, including cells with follicular T-helper cell phenotype, was most consistent with angioimmunoblastic T-cell lymphoma. The other component, revealing spindle cells forming whorls with immunostaining for CD21, CD23, and fascin, might be an integral part of this T-cell lymphoma. However, due to the often massive involvement of the nodal tissue by these follicular dendritic cells, these areas were questionably suggestive of involvement by follicular dendritic cell sarcoma...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28197347/hodgkin-lymphoma-and-castleman-disease-when-one-blood-disease-can-hide-another
#19
L Filliatre-Clement, H Busby-Venner, C Moulin, G Roth-Guepin, A Perrot
We describe a rare case of Castleman disease associated de novo with Hodgkin lymphoma. The incidence of Castleman disease is rare; only a few studies have described it in de novo association with Hodgkin lymphoma. The patient described here complained of unique evolutionary axillary adenopathy. A positron-emission tomography/computed tomography scan revealed hypermetabolic activity in this area. Diagnosis was based on a total excision biopsy of the adenopathy. The patient underwent complete remission with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) chemotherapy for treating Hodgkin lymphoma after surgical excision of the unicentric Castleman disease lesion...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28197346/allogeneic-transplant-in-elane-and-mefv-mutation-positive-severe-cyclic-neutropenia-review-of-prognostic-factors-for-secondary-severe-events
#20
Onyemaechi N Okolo, Emmanuel Katsanis, Seongseok Yun, Candace Y Reveles, Faiz Anwer
Objective and Importance. Cyclic neutropenia (CyN) is a rare autosomal dominant inherited disorder due to the mutation ELANE primarily affecting bone marrow stem cells and is characterized by recurrent neutropenia every 2 to 4 weeks. Symptoms vary from benign to severe, including death. Postulations on the cause of wide spectrum in symptom presentation include the possibility of other genetic mutations, such as MEFV. Recommended treatment for CyN is G-CSF to keep ANC higher to minimize risk of infection. Case...
2017: Case Reports in Hematology
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