Hematology Reports

4 pages, 118 KiB

Open AccessCase Report

Double Heterozygosity for Hemoglobin C and Beta-Thalassemia Dominant: A Rare Case of Thalassemia Intermedia

by Alexandra Agapidou, Paul King, Cecilia Ng and Dimitris A. Tsitsikas

Hematol. Rep. 2017, 9(4), 7447; https://doi.org/10.4081/hr.2017.7447 - 3 Jan 2018

Cited by 2 | Viewed by 434

Beta thalassemia dominant results from mutations in the β globin chain gene resulting in the production of elongated, highly unstable β globin chains. Several such mutations have been described and in a heterozygous state they may confer a phenotype more severe than that [...] Read more.

Beta thalassemia dominant results from mutations in the β globin chain gene resulting in the production of elongated, highly unstable β globin chains. Several such mutations have been described and in a heterozygous state they may confer a phenotype more severe than that of β thalassemia trait and lead to a clinical syndrome of thalassemia intermedia and its associated complications such as extramedullary hemopoiesis, bone disease, endocrinopathies and iron overload even in the absence of transfusion. In this report we present a case of double heterozygosity for HbC and β thalassemia dominant leading to a series of complications that were treated successfully once the correct diagnosis was made. Full article

4 pages, 245 KiB

Open AccessCase Report

A Rare Case of De Novo CD5⁺ Diffuse Large B-Cell Lymphoma in Leukemic Phase and Positive for CD13

by Giovanni Carulli, Eugenio Mario Ciancia, Francesco Caracciolo, Paola Sammuri, Cristiana Domenichini, Maria Immacolata Ferreri, Alessia Di Vita, Virginia Ottaviano, Martina Rousseau and Mario Petrini

Hematol. Rep. 2017, 9(4), 7437; https://doi.org/10.4081/hr.2017.7437 - 3 Jan 2018

Cited by 4 | Viewed by 324

Abstract

We report a case of de novo diffuse large B-cell lymphoma (DLBCL) in leukemic phase, positive for both CD5 and CD13. Morphologic evaluation, flow cytometric immunophenotyping, karyotyping and polymerase chain reaction studies were performed. Neoplastic lymphocytes appeared as blast-like cells, positive for CD19, [...] Read more.

We report a case of de novo diffuse large B-cell lymphoma (DLBCL) in leukemic phase, positive for both CD5 and CD13. Morphologic evaluation, flow cytometric immunophenotyping, karyotyping and polymerase chain reaction studies were performed. Neoplastic lymphocytes appeared as blast-like cells, positive for CD19, CD20, CD5, CD13, CD79a, HLADR, and with restriction for surface immunoglobulin K light chains. Rearrangement of IgH gene, BCL2/IgH translocation and complex karyotype were found. The patient was treated with RCOMP regimen and achieved complete remission. However, only one month after the first restaging of disease, the patient presented with symptoms attributable to central nervous system involvement and her clinical conditions worsened rapidly. While both CD5 expression and leukemic presentation are uncommon findings in DLBCL, positivity for CD13 is very rare. The outcome of our patient shows the poor prognosis of CD5+ DLBCL with leukemic presentation. The possible role of CD13 coexpression is discussed. Full article

3 pages, 121 KiB

Open AccessCase Report

Haploidentical Hematopoietic Stem Cell Transplantation in a Myelofibrosis Patient with Primary Graft Failure

by Cristina Tecchio, Angelo Andreini, Claudio Costantini, Alberto Zamò, Donata de Sabata, Fiorenza Aprili, Roberta Galavotti, Emanuele Guardalben and Fabio Benedetti

Hematol. Rep. 2017, 9(4), 7091; https://doi.org/10.4081/hr.2017.7091 - 3 Jan 2018

Viewed by 348

Abstract

The prognosis of patients affected by myelofibrosis (MF) is usually dismal and allogeneic hematopoietic stem cell transplantation (HSCT) remains the only cure. The number of HSCTs in MF patients has recently increased. However, a major obstacle is still represented by primary graft failure [...] Read more.

The prognosis of patients affected by myelofibrosis (MF) is usually dismal and allogeneic hematopoietic stem cell transplantation (HSCT) remains the only cure. The number of HSCTs in MF patients has recently increased. However, a major obstacle is still represented by primary graft failure (PGF). Currently there are no definitive guidelines for the treatment of PGF and a second HSCT can be performed only when an allogeneic donor is rapidly available. Herein we report on a MF patient with PGF after an unrelated HSCT, who was rescued by a non-myeloablative, unmanipulated, haploidentical HSCT that resulted in persistent engraftment and bone-marrow fibrosis regression, but not in a long-term disease control. Based on this experience we briefly review the role of different conditioning regimens and hematopoietic stem cell sources in the setting of HSCT for MF patients with PGF. The role of haploidentical donors in MF patients lacking HLAmatched relatives is also discussed. Full article

3 pages, 583 KiB

Open AccessCase Report

Dasatinib-Induced Hemorrhagic Colitis Complicated with Cytomegalovirus Infection

by Aya Nakaya, Yoshiko Azuma, Shinya Fujita, Atsushi Satake, Takahisa Nakanishi, Yukie Tsubokura, Akiko Konishi, Masaaki Hotta, Hideaki Yoshimura, Kazuyoshi Ishii, Tomoki Ito and Shosaku Nomura

Hematol. Rep. 2017, 9(4), 7415; https://doi.org/10.4081/hr.2017.7415 - 22 Dec 2017

Cited by 5 | Viewed by 349

Abstract

A 69-year-old man with chronic-phase chronic myeloid leukemia was initially treated with 100 mg dasatinib once a day. Despite a major molecular response within 9 months, he developed hemorrhagic colitis 32 months after starting dasatinib. Colonoscopy identified multiple hemorrhagic ulcers in the transverse [...] Read more.

A 69-year-old man with chronic-phase chronic myeloid leukemia was initially treated with 100 mg dasatinib once a day. Despite a major molecular response within 9 months, he developed hemorrhagic colitis 32 months after starting dasatinib. Colonoscopy identified multiple hemorrhagic ulcers in the transverse colon. The pathological findings indicated cytomegalovirus infection. Dasatinib was stopped and he was started on ganciclovir. Three months later, colonoscopy confirmed the disappearance of the hemorrhagic ulcers. Dasatinib is a second-generation tyrosine kinase inhibitor used to treat chronic myeloid leukemia. As a multi-kinase inhibitor that acts on SRC-family kinases, its broader off-target kinase-inhibitory activity may account for the adverse events of dasatinib. Although gastrointestinal bleeding is common in patients taking dasatinib, the combination of cytomegalovirus infection and hemorrhagic colitis in the absence of systemic immunodeficiency is rare. Based on this case of dasatinibinduced hemorrhagic colitis with cytomegalovirus infection, we describe a possible mechanism and effective treatment. Full article

5 pages, 654 KiB

Open AccessArticle

Expression of Some ATP-Binding Cassette Transporters in Acute Myeloid Leukemia

by Antonella Maria Salvia, Flavia Cuviello, Sabrina Coluzzi, Roberta Nuccorini, Immacolata Attolico, Sara Pasquina Pascale, Faustino Bisaccia, Michele Pizzuti and Angela Ostuni

Hematol. Rep. 2017, 9(4), 7406; https://doi.org/10.4081/hr.2017.7406 - 22 Dec 2017

Cited by 20 | Viewed by 403

Abstract

Hematopoietic cells express ATP binding cassette (ABC) transporters in relation to different degrees of differentiation. One of the known multidrug resistance mechanisms in acute myeloid leukemia (AML) is the overexpression of efflux pumps belonging to the superfamily of ABC transporters such as ABCB1 [...] Read more.

Hematopoietic cells express ATP binding cassette (ABC) transporters in relation to different degrees of differentiation. One of the known multidrug resistance mechanisms in acute myeloid leukemia (AML) is the overexpression of efflux pumps belonging to the superfamily of ABC transporters such as ABCB1, ABCG2 and ABCC1. Although several studies were carried out to correlate ABC transporters expression with drug resistance, little is known about their role as markers of diagnosis and progression of the disease. For this purpose we investigated the expression, by real-time PCR, of some ABC genes in bone marrow samples of AML patients at diagnosis and after induction therapy. At diagnosis, ABCG2 was always down-regulated, while an up regulated trend for ABCC1 was observed. After therapy the examined genes showed a different expression trend and approached the values of healthy subjects suggesting that this event could be considered as a marker of AML regression. The expression levels of some ABC transporters such as ABCC6, seems to be related to gender, age and to the presence of FLT3/ITD gene mutation. Full article

2 pages, 518 KiB

Open AccessCase Report

A Rare Cause of Pericardial Effusion and Ascites: POEMS Syndrome

by Bilal Katipoglu, Zeynep Katipoğlu, Ihsan Ates, Gokhan Yirgin, Burak Furkan Demir and Fatih Acehan

Hematol. Rep. 2017, 9(4), 7384; https://doi.org/10.4081/hr.2017.7384 - 22 Dec 2017

Cited by 2 | Viewed by 527

Abstract

POEMS syndrome is an important paraneoplastic syndrome associated with multisystem involvement. Extravascular volume overload like pericardial effusion and ascites has a broad differential diagnosis. In addition, it may be initial presentation of disease. For that reason, this case report is highlight to warn [...] Read more.

POEMS syndrome is an important paraneoplastic syndrome associated with multisystem involvement. Extravascular volume overload like pericardial effusion and ascites has a broad differential diagnosis. In addition, it may be initial presentation of disease. For that reason, this case report is highlight to warn of different forms of presentation of poems syndrome. Full article

3 pages, 557 KiB

Open AccessCase Report

PRDI-BF1 and PRDI-BF1β Isoform Expressions Correlate with Disease Status in Multiple Myeloma Patients

by Gabriele Buda, Francesca Guerrini, Sara Galimberti, Enrico Orciuolo, Simone Pacini, Elisa Mazzantini and Mario Petrini

Hematol. Rep. 2017, 9(4), 7201; https://doi.org/10.4081/hr.2017.7201 - 22 Dec 2017

Cited by 2 | Viewed by 285

Abstract

Human positive regulatory domain I binding factor 1 (PRDI-BF1 or BLIMP-1) is a transcription factor that acts as a master regulator and has crucial roles in the control of differentiation and in maintaining survival of plasma cells (PC). The PRDM1 gene, which codifies [...] Read more.

Human positive regulatory domain I binding factor 1 (PRDI-BF1 or BLIMP-1) is a transcription factor that acts as a master regulator and has crucial roles in the control of differentiation and in maintaining survival of plasma cells (PC). The PRDM1 gene, which codifies for PRDI-BF1, contains an alternative promoter capable of generating a PRDI-BF1 deleted protein (called PRDI-BF1β), which lacks 101 amino acids comprising most of the regulatory domain. PRDI-BF1β has been detected in relevant quantities especially in multiple myeloma cell lines (U266 and NCI- H929). The first aim of the study was to compare, using real time polymerase chain reaction (RT-PCR), the levels of PRDI-BF1 and PRDI-BF1β in myeloma patients and in normal human bone marrow. The second step was the examination of the expression of PRDI-BF1 and PRDI-BF1β isoform depending on disease status and treatment response. We demonstrate the correlation of PRDI-BF1 and the shorter PRDI-BF1β isoform protein levels with the clinical evolution and the management of myeloma patients. Full article

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Hematol. Rep., Volume 9, Issue 4 (December 2017) – 7 articles

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