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Hematology Reports
Volume 9
Issue 2
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Hematology Reports is published by MDPI from Volume 14 Issue 1 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.

Hematol. Rep., Volume 9, Issue 2 (June 2017) – 11 articles

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3 pages, 635 KiB  
Case Report
Hodgkin’s Lymphoma in a Man with Dilated Cardiomyopathy and Paraneoplastic Ataxia: A Therapeutical Challenge
by Elisabetta Abruzzese, Malgorzata Monika Trawinska, Achille Gaspardone, Antonino Morocutti and Paolo de Fabritiis
Hematol. Rep. 2017, 9(2), 6944; https://doi.org/10.4081/hr.2017.6944 - 28 Jun 2017
Cited by 1 | Viewed by 206
Abstract
Hodgkin’s lymphoma is a cancer of the lymphatic system. We report the case of a man with Hodgkin’s lymphoma and cardiomyopathy, for which the dilemma was whether to use the standard protocol—putting the patient at risk of worsening of heart failure, but giving [...] Read more.
Hodgkin’s lymphoma is a cancer of the lymphatic system. We report the case of a man with Hodgkin’s lymphoma and cardiomyopathy, for which the dilemma was whether to use the standard protocol—putting the patient at risk of worsening of heart failure, but giving him a good chance of full recovery—or not. The standard protocol was given and the patient made a full recovery without cardiac complications. Full article
3 pages, 543 KiB  
Case Report
BK Virus-Associated Hemorrhagic Cystitis in Patients wıth Allogeneic Hematopoietic Cell Transplantation: Report of Three Cases
by Duygu Mert, Hikmetullah Batgi, Alparslan Merdin, Sabahat Çeken, Mehmet Sinan Dal, Emre Tekgündüz, Fevzi Altuntaş and Mustafa Ertek
Hematol. Rep. 2017, 9(2), 7205; https://doi.org/10.4081/hr.2017.7205 - 26 Jun 2017
Cited by 16 | Viewed by 644
Abstract
BK virus is a human polyoma virus. It is acquired in early childhood and remains life-long latent in the genitourinary system. BK virus replication is more common in receiving immunosuppressive therapy receiving patients and transplant patients. BK virus could cause hemorrhagic cystitis in [...] Read more.
BK virus is a human polyoma virus. It is acquired in early childhood and remains life-long latent in the genitourinary system. BK virus replication is more common in receiving immunosuppressive therapy receiving patients and transplant patients. BK virus could cause hemorrhagic cystitis in patients with allogeneic stem cell transplantation. Hemorrhagic cystitis is a serious complication of hematopoietic stem cell transplantation. Hemorrhagic cystitis could cause morbidity and long stay in the hospital. Diagnosis is more frequently determined by the presence of BK virus DNA detected with quantitative or real-time PCR testing in serum or plasma and less often in urine. The reduction of immunosuppression is effective in the treatment of BK virus infection. There are also several agents with anti-BK virus activity. Cidofovir is an active agent against a variety of DNA viruses including poliomyoma viruses and it is a cytosine nucleotide analogue. Intravenous immunoglobulin IgG (IVIG) also includes antibodies against BK and JC (John Cunningham) viruses. Hereby, we report three cases of hemorrhagic cystitis. Hemorrhagic cystitis developed in all these three cases of allogeneic stem cell transplantation due to acute myeloid leukemia (AML). BK virus were detected as the cause of hemorrhagic cystitis in these patients. Irrigation of the bladder was performed. Then levofloxacin 1 × 750 mg intravenous and IVIG 0.5 gr/kg were started. But the hematuria did not decreased. In the first case, treatment with leflunomide was started, but patient died due to refractory AML and severe graft-versus-host disease after 4th day of leflunamide and levofloxacin treatments. Cidofovir treatment and the reduction of immunosuppressive treatment decreased the BK virus load and resulted symptomatic improvement in the second case. Initiation of cidofovir was planned in the third case. Administration of cidofovir together with the reduction of immunosuppression in the treatment of hemorrhagic cystitis associated with BK virus in allogeneic stem cell transplant recipients could be a good option. Full article
3 pages, 573 KiB  
Brief Report
Bridging-to-Transplant with Azacitidine for Myelodysplastic Syndrome and Acute Myeloid Leukemia, Reduces the Incidence of Acute Graft-versus-Host Disease
by Koichi Murakami, Hironori Ueno, Takashi Okabe, Toshiya Kagoo, Saigen Boku, Takahiro Yano and Akihiro Yokoyama
Hematol. Rep. 2017, 9(2), 7114; https://doi.org/10.4081/hr.2017.7114 - 15 Jun 2017
Cited by 4 | Viewed by 394
Abstract
Allogeneic stem cell transplantation (allo-SCT) is the only curative option for myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Azacitidine (AZA) has a good toxicity profile compared with intensive chemotherapy and can be considered a pre-transplant regimen in elderly patients and in patients [...] Read more.
Allogeneic stem cell transplantation (allo-SCT) is the only curative option for myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Azacitidine (AZA) has a good toxicity profile compared with intensive chemotherapy and can be considered a pre-transplant regimen in elderly patients and in patients with comorbidities. To investigate the impact of pre-transplant AZA on patient outcome after allo-SCT, we conducted a retrospective analysis of AZA pre-treatment followed by allo-SCT in patients with high-risk MDS and AML. Twenty patients who were divided into two groups according to AZA treatment given prior to allo-SCT (AZA vs non- AZA group, 10 each). Overall survival, event-free survival and incidence of chronic graft-versus-host disease (GVHD) were not significantly different between the two groups. The overall incidence of grade II to IV acute GVHD in the AZA group was significantly lower than that in the non-AZA group (p = 0.004). Bridging to transplant with AZA should be considered as an immunomodulator and effective treatment strategy for patients with MDS and AML. Full article
3 pages, 757 KiB  
Brief Report
Idiopathic Pulmonary Hemosiderosis Mimicking Iron Deficiency Anemia: A Delayed Diagnosis?
by Sultan Aydin Koker, Salih Gözmen, Yeşim Oymak, Tuba Hilkay Karapinar, Demet Can, Sinan Genç and Raziye Canan Vergin
Hematol. Rep. 2017, 9(2), 7048; https://doi.org/10.4081/hr.2017.7048 - 15 Jun 2017
Cited by 10 | Viewed by 421
Abstract
Idiopathic pulmonary hemosiderosis (IPH) is an uncommon chronic disorder in children. It is characterized by recurrent pulmonary hemorrhage and may result in hemoptysis and pulmonary insufficiency. The most common hematologic manifestation of IPH is iron deficiency anemia. The etiology of IPH is not [...] Read more.
Idiopathic pulmonary hemosiderosis (IPH) is an uncommon chronic disorder in children. It is characterized by recurrent pulmonary hemorrhage and may result in hemoptysis and pulmonary insufficiency. The most common hematologic manifestation of IPH is iron deficiency anemia. The etiology of IPH is not known and its diagnosis may be difficult due to the variable clinical courses. The most helpful signs for identifying IPH are iron deficiency anemia and recurrent or chronic cough, hemoptysis, dyspnea, wheezing. We report here 5 pediatric cases of IPH presenting with iron deficiency anemia and without pulmonary symptoms. Mean corpuscular volume was low in all patients; iron was low in 4 out of 5 cases; total iron binding capacity was high in all of them; ferritin was low in 3 patients. At follow up, none of them had responded successfully to the iron therapy. Although they didn’t present with pulmonary symptoms, chest radiographs incidentally revealed diffuse reticulonoduler shadows in all of them. Computed tomography revealed diffuse ground-glass opacities, consolidation, increased density. The diagnosis was confirmed by the detection of hemosiderinladen macrophages in bronchoalveolar lavage fluid and gastric aspirate. If patients with iron deficiency anemia don’t respond to iron therapy, they should be examined for IPH. Chest radiographs should be taken even in absence of pulmonary symptoms. Early diagnosis is important for a timely management of IPH. Full article
3 pages, 629 KiB  
Case Report
Thalidomide for the Treatment of Gastrointestinal Bleeding Due to Angiodysplasia in a Patient with Glanzmann’s Thrombasthenia
by Bruno K. L. Duarte, Silvia M. de Souza, Carolina Costa-Lima, Samuel S. Medina and Margareth C. Ozelo
Hematol. Rep. 2017, 9(2), 6961; https://doi.org/10.4081/hr.2017.6961 - 15 Jun 2017
Cited by 7 | Viewed by 373
Abstract
Angiodysplasia is a frequent cause of persistent gastrointestinal (GI) hemorrhage in elderly patients. Although GI bleeding isn’t the most common manifestation in patients with bleeding disorders, when present, it represents a challenging complication. We describe a 62-year-old patient with Glanzmann’s thrombasthenia, who used [...] Read more.
Angiodysplasia is a frequent cause of persistent gastrointestinal (GI) hemorrhage in elderly patients. Although GI bleeding isn’t the most common manifestation in patients with bleeding disorders, when present, it represents a challenging complication. We describe a 62-year-old patient with Glanzmann’s thrombasthenia, who used thalidomide for severe and recurrent GI bleeding. For 6 months, the patient experienced temporary control of GI bleeding with thalidomide in a daily oral dose of 100 mg. The anti-angiogenic effects of thalidomide have recently been explored by several groups, particularly in the management of bleeding from angiodysplasia, including cases with von Willebrand disease. Here, we review the relevant descriptions of the use of thalidomide in this situation, and also discuss potential reasons why we observed only a temporary control of the GI bleeding in our patient, such as the use of low-dose regimen due to limitations posed by thalidomide side effects. Full article
4 pages, 3804 KiB  
Case Report
Acute Myeloid Leukemia with RAM Immunophenotype: A Pediatric Case with Unusual Morphologic Features
by Miriam Conces, Rolla Abu-Arja, Suzanne Reed, Hemalatha G. Rangarajan, Terri L. Guinipero, Michael R. Loken, Lisa Eidenschink Brodersen and Samir B. Kahwash
Hematol. Rep. 2017, 9(2), 7057; https://doi.org/10.4081/hr.2017.7057 - 14 Jun 2017
Cited by 5 | Viewed by 515
Abstract
The RAM immunophenotype has been recently described as a subtype of acute myelogenous leukemia (AML) that is characterized clinically by extremely poor prognosis. We present a case of AML with RAM immunophenotype in a 5-year-old patient that resulted in poor outcome despite early [...] Read more.
The RAM immunophenotype has been recently described as a subtype of acute myelogenous leukemia (AML) that is characterized clinically by extremely poor prognosis. We present a case of AML with RAM immunophenotype in a 5-year-old patient that resulted in poor outcome despite early hematopoietic cell transplant. We describe the unusual morphologic features that, along with the distinct immunophenotype, may provide initial diagnostic clues and further justify the classification of this AML variant as a rather distinct subtype. Full article
6 pages, 658 KiB  
Review
Atypical Hemolytic Uremic Syndrome: A Brief Review
by Kuixing Zhang, Yuxin Lu, Kevin T. Harley and Minh-Ha Tran
Hematol. Rep. 2017, 9(2), 7053; https://doi.org/10.4081/hr.2017.7053 - 01 Jun 2017
Cited by 48 | Viewed by 1155
Abstract
Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur [...] Read more.
Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury. Pathologic mutations include those resulting in loss-of-function in a complement regulatory gene (CFH, CFI, CD46 or THBD) or gain-of-function in an effector gene (CFB or C3). Treatment with the late complement inhibitor, eculizumab—a monoclonal antibody directed against C5—is effective. Full article
3 pages, 582 KiB  
Case Report
Cerebral Stroke in a Teenage Girl with Paroxysmal Nocturnal Hemoglobinuria
by Francesco Gervasi, Lucia D’Amelio, Antonino Trizzino, Fabrizia Ferraro, Delia Russo, Giuseppe Santangelo, Francesca Cardella, Angela Trizzino, Floriana Di Marco and Piero Farruggia
Hematol. Rep. 2017, 9(2), 7012; https://doi.org/10.4081/hr.2017.7012 - 01 Jun 2017
Cited by 2 | Viewed by 317
Abstract
We report a case of paroxysmal nocturnal hemoglobinuria (PNH) in a 14 year-old girl presenting a cerebral arterial thrombosis. The initial diagnosis was carential anemia due to menarche following identification of slight macrocytic anemia, leucopenia and mild thrombocytopenia at routine blood analysis. The [...] Read more.
We report a case of paroxysmal nocturnal hemoglobinuria (PNH) in a 14 year-old girl presenting a cerebral arterial thrombosis. The initial diagnosis was carential anemia due to menarche following identification of slight macrocytic anemia, leucopenia and mild thrombocytopenia at routine blood analysis. The child was eventually referred to a children’s hospital after the onset of progressive fatigue, anorexia and paleness. Severe anemia (hemoglobin 6 g/dL) with negative Coombs test, mild leucopenia (white blood cells 4.9 × 109/L) and thrombocytopenia (platelets 97 × 109/L) and high values of lactate dehydrogenase (2855 U/L) were identified; a packed red cells transfusion was administered. Her condition worsened and she subsequently presented complete right hemiplegia, aphasia and coma; magnetic resonance imaging revealed a massive ischemic lesion. A diagnosis of PNH was eventually made following high sensitivity flow cytometry, which identified a PNH clone (CD66b negative equal to 93.7% of granulocytes). Fast recovery from neurologic and hematological problems occurred in response to anticoagulant therapy and intravenous therapy with eculizumab. We are convinced that PNH should be included in the differential diagnosis of children presenting with cytopenia. Full article
3 pages, 616 KiB  
Case Report
Invasive Aspergillosis with Disseminated Skin Involvement in a Patient with Acute Myeloid Leukemia: A Rare Case
by Duygu Mert, Gülşen Iskender, Fazilet Duygu, Alparslan Merdin, Mehmet Sinan Dal, Mehmet Doğan, Emre Tekgündüz, Mustafa Ertek and Fevzi Altuntaş
Hematol. Rep. 2017, 9(2), 6997; https://doi.org/10.4081/hr.2017.6997 - 01 Jun 2017
Cited by 2 | Viewed by 323
Abstract
Invasive pulmonary aspergillosis is most commonly seen in immunocompromised patients. Besides, skin lesions may also develop due to invasive aspergillosis in those patients. A 49-year-old male patient was diagnosed with acute myeloid leukemia. The patient developed bullous and zosteriform lesions on the skin [...] Read more.
Invasive pulmonary aspergillosis is most commonly seen in immunocompromised patients. Besides, skin lesions may also develop due to invasive aspergillosis in those patients. A 49-year-old male patient was diagnosed with acute myeloid leukemia. The patient developed bullous and zosteriform lesions on the skin after the 21st day of hospitalization. The skin biopsy showed hyphae. Disseminated skin aspergillosis was diagnosed to the patient. Voricanazole treatment was initiated. The patient was discharged once the lesions started to disappear. Full article
4 pages, 674 KiB  
Case Report
A Rare Case of Three Distinct Epstein-Barr Virus Associated Lymphoproliferative Disorders over Sixteen Years of Human Immunodeficiency Virus Infection
by Hamdy Mohamed Abdelaziz Ahmed, Abrar Khan, Weyman Lam, Samar Abohamad and Pradyumna Phatak
Hematol. Rep. 2017, 9(2), 6992; https://doi.org/10.4081/hr.2017.6992 - 01 Jun 2017
Viewed by 378
Abstract
Epstein Barr virus (EBV) is well known to cause different types of malignancies. In immunocompromised patients, such as those infected with human immunodeficiency virus (HIV), there is a higher likelihood of EBV related malignant transformation. Diagnosis of EBV related malignancies may be difficult [...] Read more.
Epstein Barr virus (EBV) is well known to cause different types of malignancies. In immunocompromised patients, such as those infected with human immunodeficiency virus (HIV), there is a higher likelihood of EBV related malignant transformation. Diagnosis of EBV related malignancies may be difficult and sometimes requires clinical and pathological correlation. It is very rare to have more than one type of EBV related malignancy in a single patient. Until now, there are no specific guidelines for treatment of EBV related malignancies and lymphoproliferative disorders (LPD). We present a patient who developed three different types of EBV related LPD during a sixteen-year course of HIV infection. Full article
5 pages, 593 KiB  
Article
Acute Crises and Complications of Sickle Cell Anemia among Patients Attending a Pediatric Tertiary Unit in Kinshasa, Democratic Republic of Congo
by Michel Ntetani Aloni, Bertin Tshimanga Kadima, Pépé Mfutu Ekulu, Aléine Nzazi Budiongo, René Makuala Ngiyulu and Jean Lambert Gini-Ehungu
Hematol. Rep. 2017, 9(2), 6952; https://doi.org/10.4081/hr.2017.6952 - 01 Jun 2017
Cited by 8 | Viewed by 547
Abstract
In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult [...] Read more.
In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years’ retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo. A total of 108 patients were identified as having SCA. There were 56 (51%) girls and 52 (49%) boys. Median age was 10.5 years (range 1–24 years). No child was diagnosed by neonatal screening. The median age of diagnosis of sickle cell anemia was 90 months (range: 8–250 months). The median age at the first transfusion was 36 months (range 4–168). In this series, 61 (56.5%) patients were eligible for hydroxyurea. However, this treatment was only performed in 4 (6.6%) of them. Pain episodes, acute anemic crisis and severe infection represent respectively 38.2%, 34.3% and 21.9% of events. Altered sensorium and focal deficit were encountered occasionally and represented 3.4% of acute events. Acute renal manifestations, cholelithiasis and priapism were rarely reported, in this cohort. In Kinshasa, the care of patients suffering from sickle cell anemia is characterized by the delayed diagnosis and low detection of organ complications compared to reports of Western countries. This situation is due to resources deficiencies. Full article
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