Hematology Reports

4 pages, 576 KiB

Open AccessCase Report

Idiopathic Hemophagocytic Lymphohistiocytosis during Pregnancy Treated with Steroids

by Bachar Samra, Mohamad Yasmin, Sami Arnaout and Jacques Mario Azzi

Hematol. Rep. 2015, 7(3), 6100; https://doi.org/10.4081/hr.2015.6100 - 23 Sep 2015

Cited by 24 | Viewed by 500

Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome characterized by a dysregulated hyperinflammatory immune response. The diagnosis of HLH during pregnancy is especially challenging due to the rarity of this condition. The highly variable clinical presentation, laboratory findings, and associated diagnoses [...] Read more.

Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome characterized by a dysregulated hyperinflammatory immune response. The diagnosis of HLH during pregnancy is especially challenging due to the rarity of this condition. The highly variable clinical presentation, laboratory findings, and associated diagnoses accompanying this syndrome further complicate the problem. A pronounced hyperferritinemia in the setting of systemic signs and symptoms along with a negative infectious and rheumatological workup should raise suspicions for HLH. While treatment ideally consists of immunosuppressive chemotherapy and hematopoietic stem cell transplant, the potential toxicity to both the pregnant woman and the fetus poses a challenging decision. We report the first case of idiopathic HLH presenting as fever of unknown origin in a pregnant woman successfully treated with steroids. Full article

3 pages, 564 KiB

Open AccessCase Report

Acute Liver Failure in a Pediatric Patient with Congenital Dyserythropoietic Anemia Type I Treated with Deferasirox

by Galina Ling, Vered Pinsk, Inbal Golan-Tripto and Eduard Ling

Hematol. Rep. 2015, 7(3), 5987; https://doi.org/10.4081/hr.2015.5987 - 23 Sep 2015

Cited by 7 | Viewed by 368

Abstract

Congenital dyserythropoietic anemias (CDA) represent a heterogeneous group of disorders characterized by morphological abnormalities of erythroid precursor cells and various degrees of hemolysis. Iron overload is a result of continuous hemolysis and recurrent transfusions. It is treated with iron chelators, including deferasirox. We [...] Read more.

Congenital dyserythropoietic anemias (CDA) represent a heterogeneous group of disorders characterized by morphological abnormalities of erythroid precursor cells and various degrees of hemolysis. Iron overload is a result of continuous hemolysis and recurrent transfusions. It is treated with iron chelators, including deferasirox. We present here a case of acute liver failure in a 12 years old girl with CDA type I treated with deferasirox and discuss the approach to treatment. Full article

4 pages, 685 KiB

Open AccessCase Report

Acute Non-Atherosclerotic ST-Segment Elevation Myocardial Infarction in an Adolescent with Concurrent Hemoglobin H-Constant Spring Disease and Polycythemia Vera

by Ekarat Rattarittamrong, Lalita Norasetthada, Adisak Tantiworawit, Chatree Chai-Adisaksopha, Sasinee Hantrakool, Thanawat Rattanathammethee and Pimlak Charoenkwan

Hematol. Rep. 2015, 7(3), 5941; https://doi.org/10.4081/hr.2015.5941 - 23 Sep 2015

Cited by 5 | Viewed by 285

Abstract

Thrombosis is a major complication of polycythemia vera (PV) and also a well-known complication of thalassemia. We reported a case of non-atherosclerotic ST-segment elevation myocardial infarction (STEMI) in a 17- year-old man with concurrent post-splenectomized hemoglobin H-Constant Spring disease and JAK2 V617F mutation-positive [...] Read more.

Thrombosis is a major complication of polycythemia vera (PV) and also a well-known complication of thalassemia. We reported a case of non-atherosclerotic ST-segment elevation myocardial infarction (STEMI) in a 17- year-old man with concurrent post-splenectomized hemoglobin H-Constant Spring disease and JAK2 V617F mutation-positive PV. The patient initially presented with extreme thrombocytosis (platelet counts greater than 1,000,000/μL) and three months later developed an acute STEMI. Coronary artery angiography revealed an acute clot in the right coronary artery without atherosclerotic plaque. He was treated with plateletpheresis, hydroxyurea and antiplatelet agents. The platelet count decreased and his symptoms improved. This case represents the importance of early diagnosis, awareness of the increased risk for thrombotic complications, and early treatment of PV in patients who have underlying thalassemia with marked thrombocytosis. Full article

3 pages, 544 KiB

Open AccessCase Report

Long Standing Eculizumab Treatment without Anticoagulant Therapy in High-Risk Thrombogenic Paroxysmal Nocturnal Hemoglobinuria

by Hassan A. Al-Jafar, Salma M. AlDallal, Haifa A. Askar, Ali M. Aljeraiwi and Ahmad Al-Alansari

Hematol. Rep. 2015, 7(3), 5927; https://doi.org/10.4081/hr.2015.5927 - 23 Sep 2015

Cited by 5 | Viewed by 302

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is an ultra-orphan disease affecting all hematopoietic cell types. The abnormality of red blood cells in this disease predisposes to intravascular complement-mediated hemolysis. Eculizumab is an orphan drug used to treat this rare disease. Thrombosis is the key cause [...] Read more.

Paroxysmal nocturnal hemoglobinuria (PNH) is an ultra-orphan disease affecting all hematopoietic cell types. The abnormality of red blood cells in this disease predisposes to intravascular complement-mediated hemolysis. Eculizumab is an orphan drug used to treat this rare disease. Thrombosis is the key cause of death in PNH patients in about 40% to 67% of cases. We report the case of a woman presenting with PNH complicated with serious Budd-Chiari syndrome thrombosis and with a stent inserted in the portal vein. She refused to take any anticoagulant treatment since she commenced eculizumab 4 years before. No thrombotic events happened since that time. This case could add an extra benefit for eculizumab, which could be used as an anti-thromboembolic prophylactic agent in PNH, especially in patients with thrombocytopenia, where the use of anticoagulant agents is extremely hazardous. More randomized studies might establish the use of eculizumab without anticoagulants to avoid serious bleeding that could happen in thrombocytopenic PNH patients. Full article

4 pages, 737 KiB

Open AccessCase Report

B-Cell-Rich T-Cell Lymphoma Associated with Epstein-Barr Virus-Reactivation and T-Cell Suppression Following Antithymocyte Globulin Therapy in a Patient with Severe Aplastic Anemia

by Nobuyoshi Hanaoka, Shogo Murata, Hiroki Hosoi, Aiko Shimokado, Toshiki Mushino, Kodai Kuriyama, Kazuo Hatanaka, Akinori Nishikawa, Miwa Kurimoto, Takashi Sonoki, Yasuteru Muragaki and Hideki Nakakuma

Hematol. Rep. 2015, 7(3), 5906; https://doi.org/10.4081/hr.2015.5906 - 23 Sep 2015

Cited by 4 | Viewed by 271

Abstract

B-cell lymphoproliferative disorder (B-LPD) is generally characterized by the proliferation of Epstein-Barr virus (EBV-infected B lymphocytes. We here report the development of EBV-negative B-LPD associated with EBV-reactivation following antithymocyte globulin (ATG) therapy in a patient with aplastic anemia. The molecular autopsy study showed [...] Read more.

B-cell lymphoproliferative disorder (B-LPD) is generally characterized by the proliferation of Epstein-Barr virus (EBV-infected B lymphocytes. We here report the development of EBV-negative B-LPD associated with EBV-reactivation following antithymocyte globulin (ATG) therapy in a patient with aplastic anemia. The molecular autopsy study showed the sparse EBV-infected clonal T cells could be critically involved in the pathogenesis of EBV-negative oligoclonal B-LPD through cytokine amplification and escape from T-cell surveillances attributable to ATG-based immunosuppressive therapy, leading to an extremely rare B-cell-rich T-cell lymphoma. This report helps in elucidating the complex pathophysiology of intractable B-LPD refractory to rituximab. Full article

3 pages, 616 KiB

Open AccessCase Report

A Rare Case of Myelodysplastic Syndrome with Refractory Thrombocytopenia

by Waqas Jehangir, John Webb, Shilpi Singh, Sabrina Arshed, Shuvendu Sen and Abdalla Yousif

Hematol. Rep. 2015, 7(3), 5897; https://doi.org/10.4081/hr.2015.5897 - 23 Sep 2015

Viewed by 324

Abstract

Myelodysplastic syndromes (MDS) represent a variety of clonal abnormalities, possibly preleukemic and display numerous phenotypic manifestations. Specific mutations carry high morbidity and mortality rates due to cell line dysplasia. MDS commonly presents with symptoms related to anemia, and approximately two-thirds will develop thrombocytopenia, [...] Read more.

Myelodysplastic syndromes (MDS) represent a variety of clonal abnormalities, possibly preleukemic and display numerous phenotypic manifestations. Specific mutations carry high morbidity and mortality rates due to cell line dysplasia. MDS commonly presents with symptoms related to anemia, and approximately two-thirds will develop thrombocytopenia, a rare, but potentially lethal complication that increases complexity in treatment and morbidity, and may be due to unique genetic mutations leading to refractory thrombocytopenia, ultimately leading to an overall reduction in survival. Careful identification and monitoring of this patient subdivision can significantly reduce morbidity and mortality, and potential identification of specific gene mutations and advances in treatment options will hopefully provide guidance on detecting at-risk patients in the future. We present a case of a man with MDS-U (karyotype 46, XY, del (20) (q11.2q13.3) (20) with no detected JAK2 V617F mutation), who in despite of appropriate evidenced based treatment, continued to exhibit refractory thrombocytopenia. Full article

3 pages, 548 KiB

Open AccessReview

Inflammatory Pseudotumors after Stem Cell Transplantation

by Asli Tufan and Gulistan Bahat

Hematol. Rep. 2015, 7(3), 5848; https://doi.org/10.4081/hr.2015.5848 - 23 Sep 2015

Cited by 2 | Viewed by 283

Abstract

Inflammatory pseudotumors (IPT) are rare tumors that occur in various organs and tissues. The clinical picture varies from the more frequent benign lesions to rare malignant tumors with distant metastases. IPTs associated with hematopoietic stem cell transplantation (HSCT) is rarely reported. In this [...] Read more.

Inflammatory pseudotumors (IPT) are rare tumors that occur in various organs and tissues. The clinical picture varies from the more frequent benign lesions to rare malignant tumors with distant metastases. IPTs associated with hematopoietic stem cell transplantation (HSCT) is rarely reported. In this article, we review the reports of IPT after HSCT. We also review the possible factors involved in the pathogenesis. IPT may be rare but they are a potentially serious complication of HSCT. A knowledge of these entities and insistence on a definitive biopsy of mass lesions in the post-HSCT period can avoid unnecessary treatment such as radical surgery, chemotherapy or radiotherapy. Full article

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Hematol. Rep., Volume 7, Issue 3 (September 2015) – 7 articles

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