Case Reports in Hematology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . 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. Optimal Molecular Methods in Detecting p190BCR-ABL Fusion Variants in Hematologic Malignancies: A Case Report and Review of the Literature Wed, 08 Apr 2015 08:30:20 +0000 http://www.hindawi.com/journals/crihem/2015/458052/ Patients with BCR-ABL1 positive hematologic malignancies and Philadelphia-like B-lymphoblastic leukemia (B-ALL) are potential candidates for targeted therapy with tyrosine kinase inhibitors (TKI). Before TKIs, patients with B-ALL had a much worse prognosis and current treatments with targeted TKI therapy have improved outcomes. Thus, the detection of BCR-ABL1 is crucial and a false negative BCR-ABL1 result may adversely affect patient care. We report a case of a 76-year-old male with a new diagnosis of B-ALL who was initially found to be BCR-ABL1 negative by quantitative polymerase chain reaction (PCR). A concurrent qualitative PCR was performed which detected a positive BCR-ABL1 result that was confirmed by a next generation sequencing (NGS) based assay and identified as the rare fusion variant e1a3 of p. Based on this result, the patient was placed on dasatinib as a targeted therapy. In the era of molecular diagnostic medicine and targeted therapy, it is essential to have an understanding of the limitations of molecular assays and to follow a comprehensive diagnostic approach in order to detect common abnormalities and rare variants. Incorporating NGS methods in an algorithmic manner into the standard diagnostic PCR-based approach for BCR-ABL1 will aid in minimizing false negative results. Rebecca J. Sonu, Brian A. Jonas, Denis M. Dwyre, Jeffrey P. Gregg, and Hooman H. Rashidi Copyright © 2015 Rebecca J. Sonu et al. All rights reserved. Danazol: An Effective Option in Acquired Amegakaryocytic Thrombocytopaenic Purpura Sun, 05 Apr 2015 15:57:38 +0000 http://www.hindawi.com/journals/crihem/2015/171253/ Acquired amegakaryocytic thrombocytopaenic purpura (AATP) is a rare haematological condition characterised by isolated thrombocytopaenia with normal other cell lines. It is often initially misdiagnosed as immune thrombocytopaenic purpura but has characteristic bone marrow findings of reduced megakaryocyte numbers. The optimal treatment of AATP is not clearly defined but revolves around immunosuppressive therapies. We report a case of successful treatment of AATP with danazol, an antioestrogenic medication. We also review the aetiologies and pathogenesis of the disorder and suggest that danazol should be considered as an effective alternative to potent immunosuppression in AATP. E. Mulroy, S. Gleeson, and S. Chiruka Copyright © 2015 E. Mulroy et al. All rights reserved. Localized Lymph Node Light Chain Amyloidosis Thu, 02 Apr 2015 07:06:03 +0000 http://www.hindawi.com/journals/crihem/2015/816565/ Immunoglobulin-derived light chain amyloidosis can occasionally be associated with localized disease. We present a patient with localized lymph node light chain amyloidosis without an underlying monoclonal protein or lymphoproliferative disorder and review the literature of lymph node amyloidosis discussing work-up and risk factors for systemic progression. Binod Dhakal, Alexandra M. Harrington, Michael E. Stadler, and Anita D’Souza Copyright © 2015 Binod Dhakal et al. All rights reserved. Treatment of Acquired von Willebrand Syndrome and Prevention of Bleeding Postautologous Stem Cell Transplant during Severe Pancytopenia with IVIG Thu, 02 Apr 2015 06:56:43 +0000 http://www.hindawi.com/journals/crihem/2015/809313/ The use of high dose chemotherapy followed by autologous hematopoietic stem cell transplantation for remission consolidation after initial induction represents standard of care for patients with multiple myeloma. Patients with myeloma and Acquired von Willebrand Syndrome (AVWS) undergoing autologous stem cell transplant (ASCT) are at significant risk of bleeding due to the profound thrombocytopenia, low Factor VIII levels, fever, and toxicities associated with the preparative regimen. We report a patient with AVWS associated with multiple myeloma who underwent autologous stem cell transplants as consolidation after initial induction and again at relapse. He was successfully treated with high dose intravenous immunoglobulin (IVIG) prior to each transplant with rapid resolution of AVWS. Behyar Zoghi, Paul Shaughnessy, Roger M. Lyons, Richard Helmer III, Carlos Bachier, and C. Frederick LeMaistre Copyright © 2015 Behyar Zoghi et al. All rights reserved. Two Cases of Primary Cold Agglutinin Disease Associated with Megaloblastic Anemia Mon, 30 Mar 2015 10:44:13 +0000 http://www.hindawi.com/journals/crihem/2015/913795/ We report two cases of primary cold agglutinin disease (CAD) associated with megaloblastic anemia in Japanese elderly patients. Case 1 was a 67-year-old male and Case 2 was a 55-year-old male. Both patients were diagnosed with primary CAD, with continuously high cold agglutinin titers (1 : >8,192 and 1 : 16,834, resp.), monoclonal IgM-kappa light chains, and no underlying disease. In addition, both patients had megaloblastic anemia due to vitamin B12 deficiency. One patient received rituximab and both received vitamin 12 supplementation. To date, no cooccurrence of primary CAD and megaloblastic anemia has been emphasized. Thus, the association of these hematological diseases may be incidental; however, given that CAD is an autoimmune disease which may show antibodies against intrinsic factor and gastric parietal cells, this association was thought to be probably not a coincidence. Clinicians should be aware of the possible simultaneous presence of autoimmune hemolytic/megaloblastic anemia in patients with primary CAD. Shinsaku Imashuku, Naoko Kudo, Katsushige Takagishi, and Katsuyasu Saigo Copyright © 2015 Shinsaku Imashuku et al. All rights reserved. Durable Red Blood Cell Transfusion Independence in a Patient with an MDS/MPN Overlap Syndrome Following Discontinuation of Iron Chelation Therapy Mon, 30 Mar 2015 10:00:50 +0000 http://www.hindawi.com/journals/crihem/2015/253294/ Background. Hematologic improvement (HI) occurs in some patients with acquired anemias and transfusional iron overload receiving iron chelation therapy (ICT) but there is little information on transfusion status after stopping chelation. Case Report. A patient with low IPSS risk RARS-T evolved to myelofibrosis developed a regular red blood cell (RBC) transfusion requirement. There was no response to a six-month course of study medication or to erythropoietin for three months. At 27 months of transfusion dependence, she started deferasirox and within 6 weeks became RBC transfusion independent, with the hemoglobin normalizing by 10 weeks of chelation. After 12 months of chelation, deferasirox was stopped; she remains RBC transfusion independent with a normal hemoglobin 17 months later. We report the patient’s course in detail and review the literature on HI with chelation. Discussion. There are reports of transfusion independence with ICT, but that transfusion independence may be sustained long term after stopping chelation deserves emphasis. This observation suggests that reduction of iron overload may have a lasting favorable effect on bone marrow failure in at least some patients with acquired anemias. Harpreet Kochhar, Chantal S. Leger, and Heather A. Leitch Copyright © 2015 Harpreet Kochhar et al. All rights reserved. Lymph Node Flow Cytometry as a Prompt Recognition of Ultra Early Onset PTLD: A Successful Case of Rituximab Treatment Tue, 24 Mar 2015 13:49:50 +0000 http://www.hindawi.com/journals/crihem/2015/430623/ Ultra early posttransplantation lymphoproliferative disorder (PTLD) is a rare and fatal complication after hematopoietic stem cell transplantation (HSCT). Here we report, by lymph node (LN) flowcytometry, that we early recognized ultra early PTLD after an HLA-matched sibling allo-HSCT followed by a successful treatment with anti-CD20 antibody (rituximab) in a patient in progress disease for angioimmunoblastic T-cell lymphoma (AITL). The patient was conditioned with a reduced intensity conditioning (RIC) regimen. One week after transplantation, the patient developed high fever, generalized fatigue, high Epstein-Barr virus (EBV) load, and LN enlargement. An LN lymphocyte suspension and peripheral blood flowcytometry was performed to find majority of LN lymphocytes highly expressed CD20. By highly suspicious PTLD, 4 doses of rituximab (375 mg/m2 qw) were given immediately followed by reducing and withdrawing immunosuppressant reagent. PTLD was later confirmed by pathology. The patient had good response to rituximab, showing absence of fever, reduction in LN size, and no detectable EBV-DNA. Twenty months after HSCT, the patient remains well without evidence of AITL and PTLD. The current report is one of the earliest cases of PTLD after HSCT. Taken together, by LN flowcytometry as a prompt recognition, rituximab can be an effective preemptive therapy for ultra early developed PTLD. Xiaofan Li, Nainong Li, Ting Yang, Zhizhe Chen, and Jianda Hu Copyright © 2015 Xiaofan Li et al. All rights reserved. JAK2V617F: Is It Sufficient as a Single Player in Splanchnic Venous Thrombosis? Tue, 24 Mar 2015 11:36:41 +0000 http://www.hindawi.com/journals/crihem/2015/373490/ Splanchnic venous thrombosis (SVT) includes thrombosis of the hepatic, portal, and mesenteric venous system. Myeloproliferative neoplasms (MPNs) are important factors of SVT in adults. Addition of JAK2V617F mutation in WHO criteria for diagnosis of MPNs has made this test a useful tool for diagnosis. JAK2 is an intracytoplasmic tyrosine kinase that plays a critical role in signal transduction from multiple hematopoietic factor receptors. The mutation is found frequently in patients with SVT; many such patients have no other manifestations of an MPN. Although the correlation of JAK2V617F mutation with thrombotic risk in MPNs has been shown in many studies, the impact of presence of additional thrombophilic factors in these cases is yet not known. As the management of MPNs remains highly dependent on the patient’s thrombotic risk, it is important to assess the thrombotic risk factors in detail. Here, we report two cases of JAK2V617F positive MPN who also had other thrombophilic conditions and presented with recurrent thrombosis. Pratibha Dhiman and Priyanka Saxena Copyright © 2015 Pratibha Dhiman and Priyanka Saxena. All rights reserved. Nelarabine Associated Myotoxicity and Rhabdomyolysis Wed, 18 Mar 2015 10:39:27 +0000 http://www.hindawi.com/journals/crihem/2015/825670/ Nelarabine (ara-G; Arranon; compound 506U78) is an antineoplastic purine analog used for the treatment of refractory or relapsed T-cell acute lymphoblastic leukemia (T-ALL) and T-cell lymphoblastic lymphoma (T-LBL). The drug was granted accelerated approval in October 2005 by the US Food and Drug Administration (FDA) given the efficacy (induction of complete responses) noted in 2 single-arm trials (one in pediatric setting and one in adult patient population). The main spectra of toxicities that have been reported in these clinical trials and subsequent studies are hematological and neurological. Nelarabine induced rhabdomyolysis and increased creatinine phosphokinase (CK; CPK) levels apparently have been reported and this side effect has been added as an adverse reaction in the product monograph from the drug company during postmarketing surveillance. However, the true extent and incidence of the myotoxicity from the drug are unclear. In this paper we report a grade IV CK elevation and rhabdomyolysis in a patient with T-ALL treated with nelarabine. Given the reported finding, we examined the literature further for myotoxicity, increased CK, and/or rhabdomyolysis associated with the use of the nelarabine and report our findings. Mahnur Haider, Syed Ahsan Rizvi, and Pashtoon Murtaza Kasi Copyright © 2015 Mahnur Haider et al. All rights reserved. Virus-Associated Hemophagocytic Syndrome in Renal Transplant Recipients: Report of 2 Cases from a Single Center Sun, 08 Mar 2015 14:15:10 +0000 http://www.hindawi.com/journals/crihem/2015/876301/ Virus-associated hemophagocytic syndrome (HPS) is a potentially fatal complication of immunosuppression for transplantation. However, it presents with heterogeneous clinical symptoms (fever, disturbed consciousness, and hepatosplenomegaly) and laboratory findings (pancytopenia, elevated hepatic enzyme levels, abnormal coagulation, and hyperferritinemia), impeding diagnosis. Case 1: A 39-year-old female developed fever 4 years after ABO-incompatible living-related renal transplantation. Laboratory findings revealed thrombocytopenia, elevated hepatic enzymes, Epstein-Barr virus (EBV) DNA seropositivity, and hyperferritinemia. EBV-associated HPS was confirmed by bone marrow aspiration. Steroid pulse therapy and etoposide were ineffective. Disseminated intravascular coagulation resulted in multiple organ failure, and the patient died 32 days after disease onset. Case 2: A 67-year-old male was admitted with rotavirus enteritis a month after living-unrelated renal transplantation. He developed sudden-onset high fever, disturbance of consciousness, and tachypnea 8 days after admission. Laboratory findings revealed elevated hepatic enzyme levels, hyperkalemia, and hyperferritinemia. Emergency continuous hemodiafiltration ameliorated the fever, and steroid pulse therapy improved abnormal laboratory values. Varicella-zoster virus meningitis was confirmed by spinal tap. Acyclovir improved consciousness, and he was discharged 87 days after admission. Fatal virus-associated HPS may develop in organ transplant patients receiving immunosuppressive therapy. Pathognomonic hyperferritinemia is useful for differential diagnosis. Koji Nanmoku, Takayuki Yamamoto, Makoto Tsujita, Takahisa Hiramitsu, Norihiko Goto, Akio Katayama, Shunji Narumi, Yoshihiko Watarai, Takaaki Kobayashi, and Kazuharu Uchida Copyright © 2015 Koji Nanmoku et al. All rights reserved.