Hematology Reports

4 pages, 561 KiB

Open AccessCase Report

Successful Treatment of Immune Reconstitution Inflammatory Syndrome-Related Hemophagocytic Syndrome in an HIV Patient with Primary Effusion Lymphoma

by Markela Pagonitsa Zorzou, Maria Chini, Athina Lioni, Georgios Tsekes, Thomas Nitsotolis, Ioannis Tierris, Nicolaos Panagiotou, Dimitra Rontogianni, Nicolaos Harhalakis and Marios Lazanas

Hematol. Rep. 2016, 8(4), 64-67; https://doi.org/10.4081/hr.2016.6581 - 22 Dec 2016

Cited by 5 | Viewed by 357

Abstract

Although the connection of [secondary hemophagocytic syndrome (sHS)] with HIV has been well documented, optimal treatment regimen is not well established. This is due not only to the rarity of the syndrome, but also to the heterogeneity of the involved population. Most cases [...] Read more.

Although the connection of [secondary hemophagocytic syndrome (sHS)] with HIV has been well documented, optimal treatment regimen is not well established. This is due not only to the rarity of the syndrome, but also to the heterogeneity of the involved population. Most cases are related to opportunistic infections or malignancies in advanced stage, but many cases are also related to seroconversion, in the primary infection setting. Moreover, in the [antiretroviral treatment (ART)] era, rare cases of ART-related sHS have been reported. In these, often fatal cases, an [immune reconstitution inflammatory syndrome (IRIS)] process is involved, posing a serious challenge to the treating physician. We hereby report a case of successful treatment of an HIV patient with primary effusion lymphoma who experienced sHS shortly after ART onset. Our patient, treated with high dose dexamethasone and gamma globulin, achieved complete remission. This case might hint possible therapeutic insights in the treatment of IRIS-related sHS. Full article

3 pages, 724 KiB

Open AccessCase Report

Invasive Thymoma with Pure Red Cell Aplasia and Amegakaryocytic Thrombocytopenia

by Takuya Onuki, Yusuke Kiyoki, Sho Ueda, Masatoshi Yamaoka, Seiich Shimizu and Masaharu Inagaki

Hematol. Rep. 2016, 8(4), 61-63; https://doi.org/10.4081/hr.2016.6680 - 9 Dec 2016

Cited by 8 | Viewed by 390

Abstract

We here describe a case involving a 67-yearold female patient who was referred to our hospital due to severe anemia (hemoglobin, 5.0 g/dL), thrombocytopenia (platelet count, 0.6 × 104/μL), and a mediastinal shadow with calcification noted on X-ray. On admission, an anterior mediastinal [...] Read more.

We here describe a case involving a 67-yearold female patient who was referred to our hospital due to severe anemia (hemoglobin, 5.0 g/dL), thrombocytopenia (platelet count, 0.6 × 104/μL), and a mediastinal shadow with calcification noted on X-ray. On admission, an anterior mediastinal tumor was detected, and bone marrow biopsy revealed few megakaryocytes and severely reduced numbers of erythroid cells. The diagnosis was thymoma with pure red cell aplasia (PRCA) and acquired amegakaryocytic thrombocytopenia (AAMT). On Day 8 of admission, the patient received immunosuppressive therapy together with cyclosporine for the 2 severe hematologic diseases, which were stabilized within 2 months. Subsequently, total thymectomy was performed. The diagnosis of the tumor invading the left lung was invasive thymoma, Masaoka- Koga stage III. The histological diagnosis was World Health Organization type AB. Thymoma accompanied with PRCA and AAMT is very rare, and, based on our case, immunotherapeutic therapy for the hematologic disorders should precede surgical intervention. Full article

5 pages, 590 KiB

Open AccessArticle

Study on Hydroxyurea Response in Hemoglobinopathies Patients Using Genetic Markers and Liquid Erythroid Cultures

by Serena Sclafani, Alice Pecoraro, Veronica Agrigento, Antonio Troia, Rosario Di Maggio, Massimiliano Sacco, Aurelio Maggio, Elena D'Alcamo and Rosalba Di Marzo

Hematol. Rep. 2016, 8(4), 56-60; https://doi.org/10.4081/hr.2016.6678 - 9 Dec 2016

Cited by 9 | Viewed by 464

Abstract

Increased expression of fetal hemoglobin (HbF) may ameliorate the clinical course of hemoglobinopathies. Hydroxyurea (HU) is the only inducer approved for the treatment of these diseases able to stimulate HbF production but patients’ response is highly variable indicating the utility of the identification [...] Read more.

Increased expression of fetal hemoglobin (HbF) may ameliorate the clinical course of hemoglobinopathies. Hydroxyurea (HU) is the only inducer approved for the treatment of these diseases able to stimulate HbF production but patients’ response is highly variable indicating the utility of the identification of pharmacogenomic biomarkers in order to predict pharmacological treatment efficacy. To date few studies to evaluate the role of genetic determinants in HU response have been conducted showing contradictory results. In this study we analyzed BCL11A, GATA-1, KLF-1 genes and γ-globin promoter in 60 alleles from 30 hemoglobinopathies patients under HU treatment to assess the role of these markers in HU response. We did not find any association between these genetic determinants and HU response. Before treatment started, the same patients were analyzed in vitro using liquid erythroid cultures in a test able to predict their response to HU. The results of our analysis confirm the absence of pharmacogenomic biomarker associated to HU response indicating that, the quantification of γ-globin mRNA fold increase remains the only method able to predict in vivo patients response to the drug. Full article

3 pages, 554 KiB

Open AccessCase Report

Reversible Cerebral Vasoconstriction Syndrome Promptly Diagnosed with Magnetic Resonance Imaging Including Magnetic Resonance Angiography during Immunosuppressive Therapy in a 16-Year-Old Girl with Refractory Cytopenia of Childhood

by Hideaki Ueki, Yasushi Sanayama, Akiyo Miyajima, Taichiro Tsuchimochi, Shunji Igarashi and Shosuke Sunami

Hematol. Rep. 2016, 8(4), 61-63; https://doi.org/10.4081/hr.2016.6673 - 17 Nov 2016

Cited by 8 | Viewed by 392

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is a syndrome characterized by severe headache with segmental vasoconstriction of the cerebral arteries that resolves within 12 weeks. A 16-year-old girl with refractory cytopenia of childhood, who was receiving the immunosuppressant cyclosporine, developed severe headache and was [...] Read more.

Reversible cerebral vasoconstriction syndrome (RCVS) is a syndrome characterized by severe headache with segmental vasoconstriction of the cerebral arteries that resolves within 12 weeks. A 16-year-old girl with refractory cytopenia of childhood, who was receiving the immunosuppressant cyclosporine, developed severe headache and was diagnosed with RCVS using magnetic resonance imaging, including magnetic resonance angiography (MRA). MRA is a non-invasive and very effective technique for diagnosing RCVS. MRA should be performed at the onset of severe headache during immunosuppressant administration for children with hematological disorders and may prevent sequelae such as posterior reversible encephalopathy syndrome or ischemic attack. Full article

2 pages, 551 KiB

Open AccessCase Report

Romidepsin Controls Chronic Lymphocytic Leukemia in a Patient with Mycosis Fungoides

by David M. Lemchak and Oleg E. Akilov

Hematol. Rep. 2016, 8(4), 54-55; https://doi.org/10.4081/hr.2016.6840 - 2 Nov 2016

Cited by 3 | Viewed by 267

Abstract

Romidepsin belongs to a class of medications called histone deacetylase inhibitors and is currently approved for treatment of cutaneous and peripheral T-cell lymphomas. Romidepsin was previously investigated for the treatment of chronic lymphocytic leukemia (CLL), and demonstrated potential benefit, but interest in its [...] Read more.

Romidepsin belongs to a class of medications called histone deacetylase inhibitors and is currently approved for treatment of cutaneous and peripheral T-cell lymphomas. Romidepsin was previously investigated for the treatment of chronic lymphocytic leukemia (CLL), and demonstrated potential benefit, but interest in its use declined following phase I clinical trials that showed poor tolerance of a significant side effect profile. We presented a patient with a history of stage II CLL, referred to dermatology for treatment of new-onset of mycosis fungoides (MF), who was treated with romidepsin over seven months. The patient achieved a partial response with 50% decrease in body surface area occupied by MF, thinning of remaining plaques, and near complete response in his CLL. His absolute lymphocyte count remained within the normal range for four months following discontinuation of romidepsin. Side effects were well-tolerated and did not limit therapy. Current literature on romidepsin is reviewed and compared to existing treatments for CLL. Full article

3 pages, 535 KiB

Open AccessCase Report

Treatment of Refractory Anemia with Ring Sideroblasts Associated with Marked Thrombocytosis with Lenalidomide in a Patient testing Negative for 5q Deletion and JAK2 V617F and MPL W515K/L Mutations

by Ryan Keen, Jeremy Pantin, Natasha Savage and Paul M. Dainer

Hematol. Rep. 2016, 8(4), 48-50; https://doi.org/10.4081/hr.2016.6592 - 2 Nov 2016

Cited by 7 | Viewed by 370

Abstract

Refractory anemia with ring sideroblasts associated with marked thrombocytosis (RARS-T) is a hematologic malignancy that often results in transfusion dependency and a hypercoagulable state. This rare disease currently lacks formal guidelines for treatment; however, various case reports have demonstrated efficacy in the use [...] Read more.

Refractory anemia with ring sideroblasts associated with marked thrombocytosis (RARS-T) is a hematologic malignancy that often results in transfusion dependency and a hypercoagulable state. This rare disease currently lacks formal guidelines for treatment; however, various case reports have demonstrated efficacy in the use of lenalidomide. This immunomodulatory drug has shown promise in patients with 5q deletions, with reports of achieving transfusion independence and normalization of platelet counts. Herein we present the case of a 68-year-old African American woman with RARS-T who tested negative for 5q deletion and JAK2 V617F and MPL W515K/L mutations. Her treatment with lenalidomide therapy resulted in a five-year durable complete clinical response. Full article

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Hematol. Rep., Volume 8, Issue 4 (November 2016) – 6 articles

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