Case Reports in Hematology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . All rights reserved. Distinctive Acellular Lipid Emboli in Hemoglobin SC Disease following Bone Marrow Infarction with Parvovirus Infection Thu, 27 Aug 2015 07:49:52 +0000 http://www.hindawi.com/journals/crihem/2015/328065/ An adolescent with mild hemoglobin SC disease presented with pelvic pain with subsequent respiratory and neurologic deterioration, which led to ultimately death. The autopsy demonstrated acellular fat emboli particularly in the lung and brain. There was marrow necrosis in the lumbar spine with aggregated sickle cells and positive parvovirus immunostaining. The brain lesion both grossly and microscopically presented a distinct pathology of acellular fat emboli that led to the correct diagnosis of this increasingly recognized association of sickle hemoglobinopathies with fat embolism syndrome (FES). A clinical diagnosis of FES is difficult to confirm in many patients with sickle hemoglobinopathy presenting with pain crisis because of concurrent illness. However, this case report highlights the need for a thorough knowledge of the signs and symptoms of the syndrome and a high index of suspicion for the diagnosis to be made premortem. Danielle M. Graff, Erin Owen, Robert Bendon, Salvatore Bertolone, and Ashok Raj Copyright © 2015 Danielle M. Graff et al. All rights reserved. B Cell Lymphoma, Unclassifiable, Transformed from Follicular Lymphoma: A Rare Presentation with Review of the Literature Wed, 26 Aug 2015 14:09:05 +0000 http://www.hindawi.com/journals/crihem/2015/651764/ B cell lymphoma, unclassifiable, with features of diffuse large B cell lymphoma and classical Hodgkin’s lymphoma (BCLu-DLBCL/CHL) is more commonly known as gray zone lymphoma. These cases more often present with mediastinal disease. In this report, we present a very rare case of BCLu-DLBCL/CHL without mediastinal involvement, transformed from follicular lymphoma (FL) to BCLu-DLBCL/CHL. This patient initially presented with a mass in the right neck; biopsy of the lymph node showed predominantly nodular, follicular pattern. Immunohistochemical (IHC) staining of tumor cells expressed positivity for mature B cell markers CD20, CD19, CD10, CD23, CD45, and CD38 but negative for CD5,11c. Hence, diagnosed with FL, he was given rituximab, cyclophosphamide, vincristine, and prednisone (RCVP) regimen, followed by maintenance rituximab. He showed good response. After 2 years, he presented again with a mass in the right side of the neck. Although the needle core biopsy of this mass was suggestive of B cell lymphoma, excisional biopsy showed morphological features of DLBCL as well as foci of histological pattern of CHL. IHC staining expressed positivity for CD20, CD79a, PAX5, and CD15 and CD30 consistent with DLBCL and CHL. He was diagnosed with BCLu-DLBCL/CHL. The patient received “ACVBP” (doxorubicin, cyclophosphamide, vindesine, bleomycin, and prednisone) followed by radiation. BCLu-DLBCL/CHL is clinically an aggressive tumor with poorer outcomes, but our case showed complete response to ACVBP regimen with tumor regression. Anila Kanna, Swati Agrawal, Kumar Jayant, Varun Kumar Pala, Mohammad Altujjar, Tarik Hadid, and Muhammad Khurram Copyright © 2015 Anila Kanna et al. All rights reserved. Promyelocytic Leukemia with No Retinoic Acid Receptor Alpha Abnormality but with RUNX1T1 Insertion to Chromosome 7q: A Classification and Management Dilemma Mon, 17 Aug 2015 16:48:48 +0000 http://www.hindawi.com/journals/crihem/2015/412016/ A case of acute promyelocytic leukemia (APL) with RUNX1T1 insertion to 7q is described and compared to reported cases of APL with negative retinoic acid receptor alpha (RARA) abnormality. In this report, we describe the case of a 2-year-old boy who presented with bone pain and was found to have pancytopenia. Bone marrow examination showed morphologic and immunophenotypic findings typical of APL, but conventional cytogenetics, fluorescence in situ hybridization (FISH), and real-time polymerase chain reaction (RT-PCR) showed no evidence of RARA rearrangements. The only cytogenetic abnormality found was a small insertion in 7q, and three copies of RUNX1T1. Gene sequencing results became available after initiating therapy but were not informative. We describe the rarity of such cases and discuss how the typical morphologic and immunophenotypic findings of APL, coupled with the definite absence of RARA rearrangement (by FISH and RT-PCR), present a diagnostic and classification dilemma, raising the possibility of an unknown alternative mechanism for the leukemogenesis and maturation arrest seen in other APL variants. The diagnostic challenges and urgent management issues this unusual case raises may justify including it, along with similar cases, in a separate subtype of acute myeloid leukemia (AML) in future classifications. Kathleen Overholt, Terri L. Guinipero, Nyla A. Heerema, Michael R. Loken, and Samir B. Kahwash Copyright © 2015 Kathleen Overholt et al. All rights reserved. Variant Guillain-Barré Syndrome in a Patient with Non-Hodgkin’s Lymphoma Tue, 11 Aug 2015 06:35:02 +0000 http://www.hindawi.com/journals/crihem/2015/979237/ We report a 72-year-old female patient with diffuse large B cell non-Hodgkin’s lymphoma (NHL) with previous treatment with standard chemotherapy presenting as an acute, ascending, sensorimotor polyneuropathy. Nerve conduction studies and lumbar puncture supported a rare, but ominous, axonal variant of Guillain-Barré Syndrome (GBS) known as acute motor and sensory axonal neuropathy (AMSAN), which is distinguished from the more common, acute demyelinating forms of GBS. Previous reports have largely focused on toxicities secondary to chemo- or radiotherapy as a major contributor to the development of acute neuropathies in malignancy. Clinicians should also be mindful of direct neoplastic invasion or, less commonly, paraneoplastic phenomenon, as alternative mechanisms, the latter possibly reflecting immune dysregulation in particularly aggressive lymphomas. At the time of writing, this is the first report in the literature of an axonal variant of GBS in a patient with diffuse large B cell NHL. A discussion regarding common and uncommon neuropathies in haematological malignancies is made, with a brief review of the anecdotal evidence supporting a paraneoplastic association with GBS or its variant forms in the setting of lymphoma. R. H. Bishay, J. Paton, and V. Abraham Copyright © 2015 R. H. Bishay et al. All rights reserved. Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria Tue, 11 Aug 2015 06:25:17 +0000 http://www.hindawi.com/journals/crihem/2015/908087/ Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by complement-mediated intravascular hemolysis and a prothrombotic state. Patients with PNH might have slightly increased risk of infections due to complement-associated defects subsequent to CD59 deficiency. Here, we report a rare case of a 65-year-old male patient with necrotic ulcers on both legs, where the recognition of pancytopenia and microthrombi led to the diagnosis of PNH based on FLAER (FLuorescent AERolysin) flow cytometric analysis. He was subsequently started on eculizumab therapy, with starting and maintenance doses set as per drug labelling. Progression of the patient’s leg ulcers during follow-up, with fulminant tissue destruction, purulent discharge, and necrotic patches, led to a later diagnosis of necrotizing fasciitis due to Pseudomonas aeruginosa and Klebsiella pneumonia infection. Courses of broad-spectrum antibiotics, surgical debridement, and superficial skin grafting were applied with successful effect during ongoing eculizumab therapy. This case highlights the point that it is important to maintain treatment of underlying disorders such as PNH in the presence of life-threatening infections like NF. Pusem Patir, Yakup Isik, Yigit Turk, Mehmet Can Ugur, Cengiz Ceylan, Gulnur Gorgun, Nihal Mete Gokmen, Guray Saydam, and Fahri Sahin Copyright © 2015 Pusem Patir et al. All rights reserved. De Novo CD5 Negative Blastic Mantle Cell Lymphoma Presented with Massive Bone Marrow Necrosis without Adenopathy or Organomegaly Mon, 10 Aug 2015 07:00:57 +0000 http://www.hindawi.com/journals/crihem/2015/146598/ The recent World Health Organization (WHO) classification defines mantle cell lymphoma (MCL) as a distinct entity characterized by a unique immunophenotype and a molecular hallmark of chromosomal translocation t(11;14)(q13;q32). We report an unusual case of an advanced stage of CD5 negative MCL with a blastoid variant with a massive bone marrow (BM) necrosis as an initial presenting feature, with no adenopathy or hepatosplenomegaly. The pathologic features showed blastoid variant of MCL and flow cytometry showed that the tumor cells were CD5−, CD19+, CD20+, FMC-7+, CD23−, and lambda light chain restricted. Chromosomal analysis, using karyotype and fluorescent in situ hybridization (FISH), demonstrated karyotypic abnormalities in addition to the t(11;14). Our case study may be reported as a unique case of CD5− blastic MCL with unusual presentation and findings which made the diagnosis of MCL difficult. Ghaleb Elyamany, Ali Matar Alzahrani, Eman Al Mussaed, Hassan Aljasem, Sultan Alotaibi, and Hatem Elghezal Copyright © 2015 Ghaleb Elyamany et al. All rights reserved. Refractory Epistaxis due to Severe Factor V Deficiency with Inhibitor Sun, 09 Aug 2015 12:05:40 +0000 http://www.hindawi.com/journals/crihem/2015/603402/ Factor V deficiency secondary to inhibitors is extremely rare and can be caused by a wide collection of exposures such as bovine thrombin and beta lactamase antibiotics. The management of factor V deficiency with inhibitor is a condition treated based on case reports due to the rarity of this condition. We describe a complicated case of an elderly patient with severe factor V deficiency with high inhibitor titer refractory to FEIBA (anti-inhibitor coagulation complex) treated with NovoSeven concurrently with cyclosporine immunosuppression and Rituxan. Given that there are no consensus guidelines on treatment, this case offers important insight into the therapeutic approaches that can be used to treat such patients. Elizabeth S. John, Minesh D. Patel, and Julio Hajdenberg Copyright © 2015 Elizabeth S. John et al. All rights reserved. Evading Capture by Residual Disease Monitoring: Extramedullary Manifestation of JAK2 V617F-Positive Primary Myelofibrosis After Allogeneic Stem Cell Transplantation Sun, 09 Aug 2015 08:18:41 +0000 http://www.hindawi.com/journals/crihem/2015/703457/ Monitoring of the JAK2 V617F allele burden in myeloproliferative neoplasms after allogeneic stem cell transplantation is useful to determine levels of residual disease and has the potential to detect early relapse and guide subsequent clinical intervention. A case is described of a JAK2 V617F-positive primary myelofibrosis patient who underwent allogeneic stem cell transplantation. Prospective residual disease monitoring of the peripheral blood failed to detect an extramedullary manifestation of the disease, a periorbital myeloid sarcoma, arising nearly three years after transplant. This case serves to highlight a pitfall in residual disease monitoring for myeloproliferative neoplasm-associated mutations in the post-allogeneic stem cell transplantation setting. Stephen E. Langabeer, James Nolan, Karl Haslam, Lindsey Clarke, Richard Flavin, and Eibhlin Conneally Copyright © 2015 Stephen E. Langabeer et al. All rights reserved. Metastatic Calcinosis Cutis: A Case in a Child with Acute Pre-B Cell Lymphoblastic Leukemia Wed, 05 Aug 2015 12:04:50 +0000 http://www.hindawi.com/journals/crihem/2015/384821/ Hypercalcemia in children with malignancy is an uncommon condition. It has been described in leukemia patients with impaired renal excretion of calcium or osteolytic lesions. Metastatic calcinosis cutis (MCC) may develop if hypercalcemia persists. We report the case of a 5-year-old girl with an atypical dermatosis and unspecific gastrointestinal symptoms. Considered clinical diagnoses were xanthomas, histiocytosis, molluscum contagiosum, and nongenital warts. Cutaneous histological analysis showed amorphous basophilic deposits in the dermis suggestive of calcium deposits. Laboratory tests confirmed serum hypercalcemia. Extensive investigations such as bone marrow biopsy established the diagnosis of an acute pre-B cell lymphoblastic leukemia. Hypercalcemia in hematopoietic malignancies is unusual, especially as initial manifestation of the disease. Careful review of the literature fails to reveal previous reports of these peculiar cutaneous lesions of MCC in children with leukemia. Juan Pablo Castanedo-Cázares, Amalia Reyes-Herrera, Diana Hernández-Blanco, Cuauhtémoc Oros-Ovalle, and Bertha Torres-Álvarez Copyright © 2015 Juan Pablo Castanedo-Cázares et al. All rights reserved. Melanonychia Secondary to Long-Term Treatment with Hydroxycarbamide: An Essential Thrombocytosis Case Sun, 02 Aug 2015 07:43:17 +0000 http://www.hindawi.com/journals/crihem/2015/653178/ Hydroxycarbamide is used in the treatment of essential thrombocytosis and other myeloproliferative disorders. We report the case of a 63-year-old woman with essential thrombocytosis who had melanonychia after the long-term use of the hydroxycarbamide with a dose of 1000 mg/day. Two years after the initiation of the hydroxycarbamide, our patient had pain on her toes and melanonychia on her nails. Hydroxycarbamide treatment was discontinued because of pain and she was given anagrelide treatment. The pathogenesis of melanonychia secondary to long-term hydroxycarbamide treatment is not yet well understood. Some investigators suggested that genetic factors, induction of melanocytes, and some changes in nail matrix could be the reason of hydroxycarbamide related melanonychia. Our patient has suffered color changes in her nails as well as pain that made us doubtful for a beginning of ulceration besides melanonychia. Maybe early clinical reaction of discontinuation of the drug has prevented more severe side effect like ulceration in our patient. Also side effect of hydroxycarbamide has developed more slowly in our patient compared to other patients in the mentioned study. To conclude, long-term hydroxycarbamide treatment can cause mucocutaneous side effects and more studies should be done in future in order to reveal the underlying mechanism. Umit Yavuz Malkan, Gursel Gunes, Eylem Eliacik, Okan Yayar, and Ibrahim Celalettin Haznedaroglu Copyright © 2015 Umit Yavuz Malkan et al. All rights reserved. Acute Promyelocytic Leukemia Presenting with Severe Marrow Fibrosis Sun, 12 Jul 2015 11:13:31 +0000 http://www.hindawi.com/journals/crihem/2015/826894/ We report a case of acute promyelocytic leukemia (APL) presenting with severely fibrotic marrow. There are four other reports of similar cases in the literature. Our patient was treated with All-Transretinoic Acid- (ATRA-) containing induction chemotherapy, followed by consolidation and maintenance therapy. He achieved a complete morphologic remission with adequate count recovery in a timely fashion, and later a molecular remission was documented. The patient remains in molecular remission and demonstrates normal blood counts now more than 4 years after induction. Since the morphological appearance may not be typical and the bone marrow may not yield an aspirate for cytogenetic analysis, awareness of such entity is important to make a correct diagnosis of this potentially curable disease. Harsh Shah, Carol Bradford, and Hamid Sayar Copyright © 2015 Harsh Shah et al. All rights reserved. Neonatal Acute Megakaryoblastic Leukemia Presenting with Leukemia Cutis and Multiple Intracranial Lesions Successfully Treated with Unrelated Cord Blood Transplantation Wed, 01 Jul 2015 10:56:58 +0000 http://www.hindawi.com/journals/crihem/2015/610581/ Neonatal acute megakaryoblastic leukemia (AMKL) without Down syndrome (DS) is an extremely rare disorder. We report of a one-day-old male infant without DS who developed AMKL with leukemia cutis and right facial nerve palsy. Magnetic resonance imaging of the patient’s brain revealed multiple intracranial tumors. A biopsy specimen of the skin lesion was suggestive of AMKL, but the bone marrow leukemic cells were less than 5% of the marrow nucleated cells. The skin and intracranial lesions had spontaneously regressed within one and a half months, but the patient’s anemia and thrombocytopenia gradually worsened and the leukemic cells in the bone marrow gradually increased to more than 20% of the nucleated cells. In addition, multiple intracranial lesions reappeared at 72 days of life. We diagnosed the patient with AMKL, and chemotherapy followed by unrelated cord blood transplantation after a reduced-intensity conditioning regimen resulted in sustained complete remission. At present, the patient is well, and he has demonstrated normal development for five years. Hiroshi Tsujimoto, Shinji Kounami, Yasuyuki Mitani, Takashi Watanabe, and Katsunari Takifuji Copyright © 2015 Hiroshi Tsujimoto et al. All rights reserved. Stabilisation of Laryngeal AL Amyloidosis with Long Term Curcumin Therapy Thu, 25 Jun 2015 09:56:37 +0000 http://www.hindawi.com/journals/crihem/2015/910528/ Multiple myeloma (MM), smoldering myeloma (SMM), and monoclonal gammopathy of undetermined significance (MGUS) represent a spectrum of plasma cell dyscrasias (PCDs). Immunoglobulin light chain amyloidosis (AL) falls within the spectrum of these diseases and has a mortality rate of more than 80% within 2 years of diagnosis. Curcumin, derived from turmeric, has been shown to have a clinical benefit in some patients with PCDs. In addition to a clinical benefit in these patients, curcumin has been found to have a strong affinity for fibrillar amyloid proteins. We thus administered curcumin to a patient with laryngeal amyloidosis and smoldering myeloma and found that the patient has shown a lack of progression of his disease for a period of five years. This is in keeping with our previous findings of clinical benefits of curcumin in patients with plasma cell dyscrasias. We recommend further evaluation of curcumin in patients with primary AL amyloidosis. Terry Golombick, Terrence H. Diamond, Arumugam Manoharan, and Rajeev Ramakrishna Copyright © 2015 Terry Golombick et al. All rights reserved. Extra-Axial Hematoma and Trimethoprim-Sulfamethoxazole Induced Aplastic Anemia: The Role of Hematological Diseases in Subdural and Epidural Hemorrhage Tue, 23 Jun 2015 10:01:04 +0000 http://www.hindawi.com/journals/crihem/2015/374951/ Objective and Importance. To illustrate the development of spontaneous subdural hematoma secondary to aplastic anemia resulting from the administration of trimethoprim-sulfamethoxazole. This is the first report of trimethoprim-sulfamethoxazole potentiating coagulopathy leading to any form of intracranial hematoma. Clinical Presentation. A 62-year-old female developed a bone marrow biopsy confirmed diagnosis of aplastic anemia secondary to administration of trimethoprim-sulfamethoxazole following a canine bite. She then developed a course of waxing and waning mental status combined with headache and balance related falls. CT imaging of the head illustrated a 3.7 cm 6.6 mm left frontal subdural hematoma combined with a 7.0 mm 1.7 cm left temporal epidural hematoma. Conclusion. Aplastic anemia is a rare complication of the administration of trimethoprim-sulfamethoxazole. Thrombocytopenia, regardless of cause, is a risk factor for the development of spontaneous subdural hematoma. Given the lack of a significant traumatic mechanism, this subset of subdural hematoma is more suitable to conservative management. Richard P. Menger, Rimal H. Dossani, Jai Deep Thakur, Frank Farokhi, Kevin Morrow, and Bharat Guthikonda Copyright © 2015 Richard P. Menger et al. All rights reserved. Idiopathic Intrahepatic Cholestasis as an Unusual Presentation of Hodgkin’s Disease Thu, 18 Jun 2015 08:18:33 +0000 http://www.hindawi.com/journals/crihem/2015/987860/ Intrahepatic cholestasis in the form of paraneoplastic phenomena is an uncommon presentation of Hodgkin’s lymphoma (HL). Herein we report the diagnosis of mixed type HL-related idiopathic intrahepatic cholestasis in a 73-year-old man presenting with jaundice, after the inguinal lymph node biopsy indicative of mixed cellular type HL and liver biopsy consistent with intrahepatic cholestasis, following several diagnostic interventions including surgery for suspected extrahepatic obstructive cholestasis. Our findings emphasize the value of early liver biopsy in the diagnostic algorithm along with consideration of HL-related idiopathic intrahepatic cholestasis as a diagnosis of exclusion in cholestatic jaundice of obscure origin. Hande Atalay, Banu Boyuk, Muhammet Ates, Aslan Celebi, Ismail Ekizoglu, Arzu A. Didik, and Aysenur A. Igdem Copyright © 2015 Hande Atalay et al. All rights reserved. Severe Refractory Immune Thrombocytopenia Successfully Treated with High-Dose Pulse Cyclophosphamide and Eltrombopag Tue, 09 Jun 2015 11:53:55 +0000 http://www.hindawi.com/journals/crihem/2015/583451/ Severe refractory ITP is clinically challenging and a variety of single or combination chemotherapies have been tried with limited outcome. We report a case of ITP that was unresponsive to multiple agents including high-dose steroid, IVIG, Rho(D) immune globulin, rituximab, cyclosporine, azathioprine, vincristine, mycophenolate mofetil, romiplostim, and eltrombopag; however, it achieved complete remission with combination treatment of cyclophosphamide and eltrombopag. Faiz Anwer, Seongseok Yun, Anju Nair, Yusuf Ahmad, Ravitharan Krishnadashan, and H. Joachim Deeg Copyright © 2015 Faiz Anwer et al. All rights reserved. Successful Treatment of Multifocal Histiocytic Sarcoma Occurring after Renal Transplantation with Cladribine, High-Dose Cytarabine, G-CSF, and Mitoxantrone (CLAG-M) Followed by Allogeneic Hematopoietic Stem Cell Transplantation Mon, 08 Jun 2015 12:47:14 +0000 http://www.hindawi.com/journals/crihem/2015/728260/ Histiocytic sarcoma (HS) is a rare, aggressive malignancy. Lesions previously called HS were typically non-Hodgkin lymphomas, not HS. As such, chemotherapy directed at lymphoid neoplasms was frequently successful, but it is unclear if these regimens are ideal for HS. We present a 33-year-old African gentleman who underwent sequential renal transplants for glomerulonephritis. He subsequently developed HS of the upper airway and multiple cutaneous sites. The patient received cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) followed by salvage ifosfamide, carboplatin, and etoposide (ICE) but had continuous progression of cutaneous involvement. Cladribine, high-dose cytarabine, G-CSF, and mitoxantrone (CLAG-M) yielded a partial response with near resolution of disease. Ultimately, the patient achieved a complete remission after myeloablative allogeneic hematopoietic stem cell transplant. HS occurring after solid organ transplant raises the possibility of HS as a potential posttransplant malignancy. The use of CLAG-M has not been reported in HS. In this case, histiocyte-directed chemotherapy with CLAG-M was superior to lymphoma-directed regimens. Julia Tomlin, Ryan K. Orosco, Sarah Boles, Ann Tipps, Huan-You Wang, Jacob Husseman, and Matthew Wieduwilt Copyright © 2015 Julia Tomlin et al. All rights reserved. Aromatase Inhibitor-Induced Erythrocytosis in a Patient Undergoing Hormonal Treatment for Breast Cancer Tue, 02 Jun 2015 11:26:04 +0000 http://www.hindawi.com/journals/crihem/2015/784783/ Aromatase inhibitors (AIs) are most commonly used for breast cancer patients with hormone receptor positive disease. Although the side effect profile of aromatase inhibitors is well known, including common side effects like arthralgia, bone pain, arthritis, hot flashes, and more serious problems like osteoporosis, we present a case of an uncommon side effect of these medications. We report the case of a postmenopausal woman on adjuvant hormonal therapy with anastrozole after completing definitive therapy for stage IIIB estrogen receptor-positive breast cancer, who was referred to hematology service for evaluation of persistent erythrocytosis. Primary and known secondary causes of polycythemia were ruled out. On further evaluation, we found that her erythrocytosis began after initiation of anastrozole and resolved after it was discontinued. We discuss the pathophysiology of aromatase inhibitor-induced erythrocytosis and reference of similar cases reported in the literature. Sri Lakshmi Hyndavi Yeruva, Stanley Madu Nwabudike, Onyekachi Henry Ogbonna, and Patricia Oneal Copyright © 2015 Sri Lakshmi Hyndavi Yeruva et al. All rights reserved. Improvement of Renal Function by Long-Term Sustained Eculizumab Treatment in a Patient with Paroxysmal Nocturnal Hemoglobinuria Mon, 01 Jun 2015 13:10:09 +0000 http://www.hindawi.com/journals/crihem/2015/673195/ Chronic kidney disease (CKD) is one of the major manifestations of paroxysmal nocturnal hemoglobinuria (PNH). CKD in PNH is induced mainly by intravascular hemolysis of PNH-affected red blood cells (RBC) missing the glycosylphosphatidylinositol-anchored proteins with complement-regulatory activities, CD55 and CD59. CKD develops by heme absorption in the proximal tubules resulting in the interstitial deposition of iron in the kidneys. We administered eculizumab to a patient with PNH, who was one of 29 patients enrolled in the AEGIS clinical trial, an open-label study of eculizumab in Japan. The patient was complicated by stage 3 CKD with impaired estimated glomerular filtration rate (eGFR), at grade G3b, and had obvious proteinuria (2-3+, 1-2 g/day). In a two-year extension to the 12-week AEGIS study, eGFR improved significantly, and the eGFR has since been maintained at grade G2 without proteinuria by sustained eculizumab treatment (>6 years). Renal function improved and maintained by long-term sustained eculizumab treatment, presumably by clearance of iron from the kidney as well as inhibition of the production of anaphylatoxin C5a, even in advanced stages of CKD, is one of the benefits of eculizumab treatment in PNH. Haruhiko Ninomiya, Naoshi Obara, Akiko Niiori-Onishi, Yasuhisa Yokoyama, Mamiko Sakata-Yanagimoto, Yuichi Hasegawa, and Shigeru Chiba Copyright © 2015 Haruhiko Ninomiya et al. All rights reserved. Cardiopulmonary Failure Requiring ECMO Bypass Resulting from Leukemia Cell Lysis in a Patient with Childhood Acute Myelomonocytic Leukemia Mon, 01 Jun 2015 09:22:40 +0000 http://www.hindawi.com/journals/crihem/2015/640528/ Background. Childhood AML patients are at increased risk for early fatal pulmonary complications. Pulmonary leukostasis and systemic inflammatory response syndrome (SIRS) following leukemia cell lysis are the likely etiologies. Observation. Soon after initiation of AML chemotherapy, an 18-month-old female who met SIRS criteria sustained cardiopulmonary failure requiring ECMO support. Upon recovery, the patient went on to complete therapy and remains in remission without permanent neurologic or cardiac sequelae. Conclusion. Cytokine release syndrome from rapid cell lysis was the likely cause as infectious workup failed to reveal a definitive etiology and drug hypersensitivity testing to the chemotherapy agents was negative. Michael Huang, Erin Owen, Scott Myers, and Ashok Raj Copyright © 2015 Michael Huang et al. All rights reserved. A Case of Fulminant Hepatitis due to Echovirus 9 in a Patient on Maintenance Rituximab Therapy for Follicular Lymphoma Thu, 28 May 2015 07:30:40 +0000 http://www.hindawi.com/journals/crihem/2015/454890/ Rituximab is a CD20 monoclonal antibody commonly used in the treatment of haematological malignancies. It causes lymphopenia with subsequent compromised humoral immunity resulting in an increased risk of infection. A number of infections and viral reactivations have been described as complicating Rituximab therapy. We report an apparently unique case of echovirus 9 (an enterovirus) infection causing an acute hepatitis and significant morbidity in an adult patient on maintenance treatment of Rituximab for follicular lymphoma. We also describe potential missed opportunities to employ more robust screening for viral infections which may have prevented delays in the appropriate treatment and thus may have altered the patient’s clinical course. We also make suggestions for lowering the threshold of viral testing in similar patients in the future. Ceri Morgan, S. J. Thomson, Joanne Legg, and Santosh Narat Copyright © 2015 Ceri Morgan et al. All rights reserved. Localized Relapse of Primary Plasma Cell Leukaemia in the Central Nervous System Sun, 10 May 2015 11:20:39 +0000 http://www.hindawi.com/journals/crihem/2015/273565/ Primary plasma cell leukaemia (pPCL) is a rare and aggressive form of plasma cell malignancies with a very poor prognosis. Compared to other plasma cell malignancies the tendency to extramedullary spread is increased; however central nervous system (CNS) involvement is rare and only reported in few cases. We report the case of a 61-year-old man who was diagnosed with pPCL and achieved a complete remission after autologous stem cell transplantation but had a relapse in the CNS without systemic disease. Christian W. Eskelund and Niels Frost Andersen Copyright © 2015 Christian W. Eskelund and Niels Frost Andersen. All rights reserved. AL Amyloidosis Complicated by Persistent Oral Bleeding Thu, 07 May 2015 06:56:13 +0000 http://www.hindawi.com/journals/crihem/2015/981346/ A case of amyloid light chain (AL) amyloidosis is presented here with uncontrolled bleeding after a nonsurgical dental procedure, most likely multifactorial in nature, and consequently treated with a multidisciplinary approach. Luiz Antonio Liarte Marconcini, Forrest Marc Stewart, Lisa Sonntag, Emily Stevens, and Nicholas Burwick Copyright © 2015 Luiz Antonio Liarte Marconcini et al. All rights reserved. Calreticulin Mutated Essential Thrombocythemia Presenting as Acute Coronary Syndrome Wed, 29 Apr 2015 08:31:21 +0000 http://www.hindawi.com/journals/crihem/2015/161764/ Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by a clonal expansion of megakaryocytes. ET can result in both arterial and venous thrombosis. Involvement of the coronary arteries has been reported. Patients who harbor a CALR mutation are half as likely to suffer a thrombotic event as compared to patients with a JAK2 mutation. We report a case of CALR-mutated ET whose initial disease manifestation was a non-ST segment elevation myocardial infarction. Bassel Nazha, Gwenalyn Garcia, Ruben Kandov, and Marcel Odaimi Copyright © 2015 Bassel Nazha et al. All rights reserved. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks Tue, 28 Apr 2015 12:47:05 +0000 http://www.hindawi.com/journals/crihem/2015/780159/ Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA) had been suffering from recurrent acute chest syndrome (ACS). Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS. Burcu Camcıoğlu, Meral Boşnak-Güçlü, Müşerrefe Nur Karadallı, Şahika Zeynep Akı, and Gülsan Türköz-Sucak Copyright © 2015 Burcu Camcıoğlu et al. All rights reserved. A Pelvic Pseudotumor in a Nonhemophilic Patient: An Unusual Presentation Mon, 27 Apr 2015 13:03:58 +0000 http://www.hindawi.com/journals/crihem/2015/359735/ Hemophilic pseudotumor is a rare complication of hemophilia, occurring in 1 to 2 percent of individuals with severe factor VIII or factor IX deficiency. A 35-year-old male presented with a swelling in the right lower abdomen for 3 months. There was no history of trauma. Examination revealed a swelling over the right iliac fossa. Right hip showed 30° flexion deformity. Blood investigations like complete blood count, APTT, PT, bleeding and clotting time, and fibrinogen were all normal. Plain radiograph and MRI showed a lytic lesion in the right iliac wing. Excision biopsy of the swelling showed organized hematoma with a fibrous capsule suggestive of a pseudotumor. Further haematological workup like factors VIII and IX was normal. At 2 years follow-up, there was no recurrence. We report this case of pseudotumour in patient without any bleeding disorder. Such case has not been reported in literature to the best of our knowledge. Mohamad Gouse, Abel Livingston, Dan Barnabas, and Vinoo Mathew Cherian Copyright © 2015 Mohamad Gouse et al. All rights reserved. Primary Lymphoma of Bone Presenting as Spindle Cell Neoplasm of the Vertebral Body: A Case Report and Review of the Literature Thu, 23 Apr 2015 11:22:11 +0000 http://www.hindawi.com/journals/crihem/2015/518307/ Spindle cell variant of lymphoma is a very rare but known disease entity that can mimic a sarcoma. Diagnosis can be even more challenging if the only site of the disease is in the bone. We report a case of primary lymphoma of bone with spindle cell morphology which was successfully treated with a combination of surgery, chemotherapy, and radiotherapy. Mahakit Inklab, Richard H. Steingart, and Jonathan K. Freeman Copyright © 2015 Mahakit Inklab et al. All rights reserved. Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease Sun, 19 Apr 2015 17:01:28 +0000 http://www.hindawi.com/journals/crihem/2015/703803/ von Willebrand disease type 3 (VWD3) is a rare but the most severe form of von Willebrand disease; it is due to almost complete lack of von Willebrand factor activity (VWF:RCo). It is inherited as autosomal recessive trait; whilst heterozygote carriers have mild, or no symptoms, patients with VWD3 show severe bleeding symptoms. In the laboratory, this is characterised by undetectable VWF:Ag, VWF:RCo, and reduced levels of factor VIII < 0.02 IU/dL. The bleeding is managed with von Willebrand/FVIII factor concentrate replacement therapy. In this rare but challenging case we report on the successful excision and repair of an ascending aortic aneurysm following adequate VWF/FVIII factor concentrate replacement using Haemate-P. Victoria Campbell, Kevin Marriott, Rex Stanbridge, and Abdul Shlebak Copyright © 2015 Victoria Campbell et al. All rights reserved. Nonsecretory Multiple Myeloma Presenting as an Intestinal Tumor Thu, 16 Apr 2015 06:33:15 +0000 http://www.hindawi.com/journals/crihem/2015/818715/ We report a case of a 43-year-old Caucasian man who presented with colicky abdominal pain and microcytic hypochromic anemia. The patient underwent a colonoscopy where a tumor was seen in the ascending colon; histology showed plasmacytoma of the colon. From the protein electrophoresis, no monoclonal band or free light chains were detected nor was urinary Bence Jones protein present. A bone marrow biopsy showed plasma cell myeloma. To the best of our knowledge, this is the first case of nonsecretory multiple myeloma presenting as plasmacytoma of the colon. Diana Triantafyllopoulou, Stuart Mellor, Catherine Cargo, Ioannis Gkikas, Jagdish Adiyodi, Ayub Ali Bin, Neil Sahasrabudhe, and Margaret Rokicka Copyright © 2015 Diana Triantafyllopoulou et al. All rights reserved. Rare Form of Erdheim-Chester Disease Presenting with Isolated Central Skeletal Lesions Treated with a Combination of Alfa-Interferon and Zoledronic Acid Wed, 08 Apr 2015 12:46:51 +0000 http://www.hindawi.com/journals/crihem/2015/876752/ Erdheim-Chester disease (ECD) represents a clonal non-Langerhans histiocytosis, which manifests under an extensive variety of clinical symptoms. This creates a challenge for the physician, who is required to recognize and diagnose the disease in the early stages. Despite this considerable challenge, in the last decade there has been a dramatic increase in ECD diagnoses, in most part due to an increasing awareness of this rare disorder. Involvement of the axial skeleton is exclusively uncommon with no official recommendations for the treatment of the bone lesions. Here, we present a case report of a young male patient with isolated lesions of the spine, ribs, and pelvis, who was successfully treated with a combination therapy of alfa-interferon and zoledronic acid. E. N. Bulycheva, V. V. Baykov, M. I. Zaraĭskiĭ, and G. N. Salogub Copyright © 2015 E. N. Bulycheva et al. All rights reserved.