Case Reports in Hematology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. Wiskott-Aldrich Syndrome Presenting with JMML-Like Blood Picture and Normal Sized Platelets Tue, 31 May 2016 09:19:12 +0000 http://www.hindawi.com/journals/crihem/2016/8230786/ Objective. The aim of this paper is to report the case of Wiskott-Aldrich syndrome (WAS) that presented with unusual laboratory features. Clinical Presentation and Intervention. Male neonate admitted with symptoms related to thrombocytopenia, whose initial diagnosis was considered as neonatal alloimmune thrombocytopenia and JMML (juvenile myelomonocytic leukemia) but subsequently diagnosis was confirmed as WAS. Conclusion. This case shows that a suspicion of WAS is warranted in the setting of neonatal thrombocytopenia with JMML-like blood picture and normal sized platelets. Rajesh B. Patil, Chandrakala Shanmukhaiah, Farah Jijina, Shailesh Bamborde, Nilesh Wasekar, Manoj Toshniwal, Aniket Mohite, and Vinod Patil Copyright © 2016 Rajesh B. Patil et al. All rights reserved. Renal Thrombotic Microangiopathy Associated with the Use of Bortezomib in a Patient with Multiple Myeloma Mon, 16 May 2016 13:40:08 +0000 http://www.hindawi.com/journals/crihem/2016/6020691/ Bortezomib is a first-generation proteasome inhibitor used in the treatment of multiple myeloma (MM). A few reports have linked bortezomib exposure with the development of thrombotic microangiopathy (TMA). We describe a case of biopsy-proven renal thrombotic microangiopathy associated with the use of bortezomib in a 51-year-old man with IgG lambda MM. To our knowledge, this is the first biopsy-proven case. In addition, reexposure to bortezomib 18 months later was associated with recurrence of TMA. This supports a possible causal role of bortezomib. The exact mechanisms remain to be elucidated. Jan Van Keer, Michel Delforge, Daan Dierickx, Kathelijne Peerlinck, Evelyne Lerut, and Ben Sprangers Copyright © 2016 Jan Van Keer et al. All rights reserved. Paraneoplastic Syndrome in Splenic Marginal Zone Lymphoma: A Rare Phenomenon of Paraplegia as an Atypical Presenting Manifestation Wed, 11 May 2016 11:12:27 +0000 http://www.hindawi.com/journals/crihem/2016/7034167/ We describe a case presenting complaint of complete lower body paraparesis, which was discovered to have splenic marginal zone lymphoma (SMZL). While paraneoplastic syndromes are more common in tumors, such as small cell lung cancer, very few reports exist on this condition with SMZL. We describe such a rare entity with a clinical course spanning twenty-four months after diagnosis. Jessica Schering and Vijayalakshmi Donthireddy Copyright © 2016 Jessica Schering and Vijayalakshmi Donthireddy. All rights reserved. Primary MALT Lymphoma of the Breast Treated with Definitive Radiation Tue, 10 May 2016 09:48:03 +0000 http://www.hindawi.com/journals/crihem/2016/1831792/ We are reporting a case of a 59-year-old woman, with a family history of breast cancer, who presented with extranodal marginal zone lymphoma (MALT) of the left breast. She received definitive radiation therapy and remains without evidence of disease. Here, we present a case and review the current literature to determine the optimal treatment of this rare presentation of MALT. Mohammad Hissourou III, Sayyad Yaseen Zia, Mahfood Alqatari, James Strauchen, and Richard L. Bakst Copyright © 2016 Mohammad Hissourou III et al. All rights reserved. Bing-Neel Syndrome Case Report: A Previously Undocumented IgG Variant with MRI, PET/CT, and PET/MRI Imaging Wed, 06 Apr 2016 13:39:36 +0000 http://www.hindawi.com/journals/crihem/2016/3931709/ Waldenstrom’s macroglobulinaemia is the most commonly reported subtype of lymphoplasmacytic lymphoma (LPL); it is characterised by IgM secretion. Neurological complications are common usually as a result of hyperviscosity. In rare cases, cells can infiltrate the central nervous system; this is known as Bing-Neel syndrome. We report the case of a 57-year-old male with lymphoplasmacytic lymphoma of the IgG-subtype with neurological symptoms and the consequent finding of lymphoplasmacytoid cells in his cerebrospinal fluid as well as deposits on MRI and PET-CT imaging. This is the first report of Bing-Neel syndrome in IgG-subtype LPL. We discuss the biological and radiological markers of his disease, including PET imaging, which has been minimal in this area to date. Daniel Halperin, Simon Hallam, Athar Haroon, Tom Butler, and Samir Agrawal Copyright © 2016 Daniel Halperin et al. All rights reserved. Marked Improvement in Refractory TTP Directly after H. pylori Eradication Therapy Tue, 05 Apr 2016 11:54:00 +0000 http://www.hindawi.com/journals/crihem/2016/1568586/ Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder involving thrombotic microangiopathy and is characterized by increased platelet aggregation throughout the body. Acquired TTP can be triggered by a variety of conditions including infections. We hereby describe a case report of an 81-year-old female presenting to the internal medicine department with TTP and active chronic gastritis, positive for Helicobacter pylori (H. pylori) on biopsy. The TTP was highly resistant to medical therapy; however the patient underwent complete resolution of her TTP following H. pylori eradication. We conclude that acquired TTP may be triggered by H. pylori infection and that treating the underlying infection may play a role in improving TTP’s outcome in some patients, especially when disease is refractory to medical therapy. Irina Gringauz, Narin Nard Carmel-Neiderman, Tobin Mangel, Orith Portnoy, Gad Segal, and Idan Goren Copyright © 2016 Irina Gringauz et al. All rights reserved. Complete Remission of Acute Myeloid Leukemia following Cisplatin Based Concurrent Therapy with Radiation for Squamous Cell Laryngeal Cancer Sun, 03 Apr 2016 16:53:54 +0000 http://www.hindawi.com/journals/crihem/2016/8581421/ Acute myeloid leukemia (AML) is a myeloid disorder with several established treatment regimens depending on patient and leukemic factors. Cisplatin is known to have strong leukemogenic potential and is rarely used even as salvage therapy in relapsed or refractory AML. We present a patient simultaneously diagnosed with AML and squamous cell carcinoma of the larynx, who was found to be in complete remission from AML following treatment with cisplatin based chemoradiotherapy for his laryngeal cancer. Mayur D. Mody, Harpaul S. Gill, Kristin A. Higgins, Nabil F. Saba, and Vamsi K. Kota Copyright © 2016 Mayur D. Mody et al. All rights reserved. Pregnancy and Accelerated Phase of Myeloid Chronic Leukemia Treated with Imatinib: A Case Report from a Developing Country Thu, 31 Mar 2016 06:51:10 +0000 http://www.hindawi.com/journals/crihem/2016/6104948/ Background. Chronic myeloid leukemia is a hematological malignancy caused by expression of BCR-ABL tyrosine kinase oncogene, product of the t(9;22) Philadelphia translocation. Accelerated phase of this disease marks the onset of advanced rapidly progressive disease unresponsive to many therapies. Pregnancy limits broad number of therapies on patients because of their potential teratogenic effects. We report the case of a pregnant 34-year-old patient on accelerated phase successively managed by imatinib. She achieved a safe pregnancy and delivered at 39 weeks a healthy baby without congenital abnormalities. Our case is unusual because of the accelerated phase of the disease. Case Presentation. A 34-year-old African female with history of chronic phase of myeloid leukemia on imatinib, lost to follow-up for 4 months, presented to the hematological department for abdominal discomfort. Accelerated phase of chronic myeloid leukemia was diagnosed. Complete hematological response was achieved on high doses of imatinib. At the completion of 39 weeks, she delivered a healthy child without congenital anomalies. Conclusion. Despite its teratogenic and embryotoxic effects, front line imatinib is the only effective, well-tolerated treatment for patient on accelerated phase that can be offered to patients in sub-Saharan countries. Lydie Ocini Ngolet, Innocent Kocko, and Alexis Elira Dokekias Copyright © 2016 Lydie Ocini Ngolet et al. All rights reserved. Extra-Adrenal Myelolipoma Containing Small Lymphocytic Lymphoma/Chronic Lymphocytic Leukemia: A Case Report and Review of the Literature Tue, 29 Mar 2016 12:24:05 +0000 http://www.hindawi.com/journals/crihem/2016/7364951/ Myelolipoma is a benign tumor consisting of mature fat interspersed with hematopoietic elements resembling bone marrow. The vast majority occurs within the adrenal glands, but several cases of extra-adrenal myelolipomas (EAMLs) have been reported. We report a case of a 64-year-old male who presented with complaint of lower abdominal discomfort. CT scan of abdomen and pelvis showed a 6 cm × 5 cm, well-circumscribed, predominantly fatty mass in the presacral region. Histological examination of the pelvic mass revealed a myelolipoma heavily infiltrated by small lymphoid cell aggregates with immunophenotypic features of small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL). Review of the literature revealed that there is only one published report of SLL/CLL involving a myelolipoma, which was also an extra-adrenal myelolipoma, and, therefore, our case is the second case of a SLL/CLL involving a myelolipoma that is an extra-adrenal myelolipoma. Extra-adrenal myelolipomas seem to the preferred myelolipomas for involvement by SLL/CLL. Komal Arora and Jagmohan Sidhu Copyright © 2016 Komal Arora and Jagmohan Sidhu. All rights reserved. Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy Tue, 22 Mar 2016 11:52:10 +0000 http://www.hindawi.com/journals/crihem/2016/9181698/ Warm autoimmune hemolytic anemia (wAIHA) is the most common form of AIHA, with corticosteroids in first-line treatment resulting in a 60–80% response rate. Atypical wAIHA and IgG plus complement mediated disease have a higher treatment failure rate and higher recurrence rate. We report a case of severe wAIHA secondary to Waldenström macroglobulinemia with life threatening intravascular hemolysis refractory to prednisone, rituximab, splenectomy, and plasmapheresis. A four-week treatment of eculizumab in this heavily pretreated patient resulted in a sustained increase in hemoglobin and transfusion independence, suggesting a role for complement inhibition in refractory wAIHA. Kim Ma and Stephen Caplan Copyright © 2016 Kim Ma and Stephen Caplan. All rights reserved. Pure White Cell Aplasia and Necrotizing Myositis Thu, 17 Mar 2016 11:58:41 +0000 http://www.hindawi.com/journals/crihem/2016/4161679/ Pure white cell aplasia (PWCA) is a rare hematologic disorder characterized by the absence of neutrophil lineages in the bone marrow with intact megakaryopoiesis and erythropoiesis. PWCA has been associated with autoimmune, drug-induced, and viral exposures. Here, we report a case of a 74-year-old female who presented with severe proximal weakness without pain and was found to have PWCA with nonspecific inflammatory necrotizing myositis and acute liver injury on biopsies. These findings were associated with a recent course of azithromycin and her daily use of a statin. Myositis improved on prednisone but PWCA persisted. With intravenous immunoglobulin and granulocyte-colony stimulating factor therapies, her symptoms and neutrophil counts improved and were sustained for months. Peter Geon Kim, Joome Suh, Max W. Adelman, Kwadwo Oduro, Erik Williams, Andrew M. Brunner, and David J. Kuter Copyright © 2016 Peter Geon Kim et al. All rights reserved. Cytogenetically Unrelated Clones in Acute Myeloid Leukemia Showing Different Responses to Chemotherapy Thu, 10 Mar 2016 08:40:54 +0000 http://www.hindawi.com/journals/crihem/2016/2373902/ We report a case of acute myeloid leukemia (AML) with two cytogenetically unrelated clones. The patient was a 45-year-old male who was diagnosed with acute monoblastic leukemia (AMoL). Initial G-band analysis showed 51,XY,+6,+8,inv(9)(p12q13)c,+11,+13,+19[12]/52,idem,+Y[8], but G-band analysis after induction therapy showed 45,XY,-7,inv(9)(p12q13)c[19]/46,XY,inv(9)(p12q13)c[1]. Retrospective FISH analysis revealed a cryptic monosomy 7 clone in the initial AML sample. The clone with multiple trisomies was eliminated after induction therapy and never recurred, but a clone with monosomy 7 was still detected in myelodysplastic marrow with a normal blast percentage. Both clones were successfully eliminated after related peripheral blood stem cell transplantation, but the patient died of relapsed AML with monosomy 7. We concluded that one clone was de novo AMoL with chromosome 6, 8, 11, 13, and 19 trisomy and that the other was acute myeloid leukemia with myelodysplasia-related changes (AML-MRC) with chromosome 7 monosomy showing different responses to chemotherapy. Simultaneous onset of cytogenetically unrelated hematological malignancies that each have a different disease status is a rare phenomenon but is important to diagnose for a correct understanding of the disease status and for establishing an appropriate treatment strategy. Kohei Kasahara, Masahiro Onozawa, Naohiro Miyashita, Emi Yokohata, Miho Yoshida, Minoru Kanaya, Mizuha Kosugi-Kanaya, Ryo Takemura, Shojiro Takahashi, Junichi Sugita, Akio Shigematsu, Mutsumi Takahata, Shinichi Fujisawa, Daigo Hashimoto, Katsuya Fujimoto, Tomoyuki Endo, Takeshi Kondo, and Takanori Teshima Copyright © 2016 Kohei Kasahara et al. All rights reserved. Simeprevir and Sofosbuvir Combination Treatment in a Patient with HCV Cirrhosis and HbS Beta 0-Thalassemia: Efficacy and Safety despite Baseline Hyperbilirubinemia Wed, 02 Mar 2016 06:08:57 +0000 http://www.hindawi.com/journals/crihem/2016/7635128/ Hyperbilirubinemia is an adverse reaction of simeprevir (SMV). The majority of these patients were taking concurrent ribavirin presenting elevated unconjugated hyperbilirubinemia due to hemolysis. However, cases of hepatic failure with elevated bilirubin level have also been reported in patients with decompensated cirrhosis. We describe a 51-year-old female patient with HbS beta 0-thalassemia and recently diagnosed compensated cirrhosis due to chronic hepatitis C infection. Laboratory evaluation revealed total bilirubin: 2.7 mg/dL and serum HCV-RNA 137.204 IU/mL. HCV was genotyped as 4. A FibroScan revealed 35.3 kPa. She was considered as illegible for pegylated-interferon-free treatment with direct acting antivirals and a course with simeprevir and sofosbuvir (SOF) combination for twelve weeks was planned. Hyperbilirubinemia developed from the beginning with peak values during the 3rd month of treatment. However, no findings of liver decompensation were noticed. Hyperbilirubinemia was benign and fully reversible and our patient finally achieved sustained virological response 24 weeks after the end of treatment. Nikolaos Papadopoulos, Melanie Deutsch, Athanasios Georgalas, Helias Poulakidas, and Lazaros Karnesis Copyright © 2016 Nikolaos Papadopoulos et al. All rights reserved. Erythrocytosis due to PHD2 Mutations: A Review of Clinical Presentation, Diagnosis, and Genetics Mon, 29 Feb 2016 11:10:45 +0000 http://www.hindawi.com/journals/crihem/2016/6373706/ The association of mutations in the PHD2 protein of the hypoxia-sensing pathway and erythrocytosis has only been established in the last decade. Here we report the case of a novel PHD2 gene mutation in a patient with erythrocytosis and summarize all reported cases to date. Case Report. A 55-year-old man presented with dyspnea and a previous diagnosis of idiopathic erythrocytosis. PHD gene sequencing revealed a mutation on exon 2. The mutation was recognized as p.(Trp) (c. 1001G>A) resulting in a truncation of a highly conserved amino acid residue in catalytic domain. A diagnosis of erythrocytosis secondary to mutant PHD2 gene was made. Conclusions. Our findings indicate that with PHD2 mutations there is moderate erythrocytosis and erythropoietin (Epo) levels are generally low to normal. Two patients with PHD2 substitution mutations were found to have paraganglioma and one of these patients had a concurrent pheochromocytoma. In addition, one mutation was associated with sagittal sinus thrombosis. Given the severity of some of the clinical features of these mutations, we conclude that clinical guidelines should include the PHD2 mutation in the idiopathic erythrocytosis workup. Rachel Wilson, Nausheen Syed, and Prabodh Shah Copyright © 2016 Rachel Wilson et al. All rights reserved. An Unusual Presentation of Pseudothrombotic Microangiopathy in a Patient with Autoimmune Atrophic Gastritis Thu, 25 Feb 2016 08:29:02 +0000 http://www.hindawi.com/journals/crihem/2016/1087831/ Introduction. We hereby describe the case of a young female patient who presented with pseudothrombotic microangiopathy, as well as pancytopenia accompanied by autoimmune atrophic gastritis. Case Presentation. A 36-year-old Caucasian woman presented to the emergency department with fatigue and dyspnea on minimal exertion. Physical examination was unremarkable except for pallor and noninjected conjunctiva. Laboratory tests revealed high LDH and low hemoglobin, white blood cells, platelets, and haptoglobin. The peripheral blood smear showed schistocytes suggestive of pseudothrombotic microangiopathy. Low cobalamin level and hyperhomocysteinemia were also detected. Autoimmune atrophic gastritis was confirmed by gastric biopsy and positive anti-intrinsic factor antibodies. Vitamin B12 supplements were given which led to rapid recovery and normalization of blood parameters. Conclusion. This case highlights the importance and serves as a reminder to clinicians to rule out cobalamin deficiency and autoimmune atrophic gastritis in patients presenting with a picture suggestive of thrombotic thrombocytopenic purpura and pancytopenia, which was completely reversible after appropriate replacement therapy without recurring to unnecessary and invasive procedures such as plasma exchange. Alexandre Malek and Roy Nasnas Copyright © 2016 Alexandre Malek and Roy Nasnas. All rights reserved. Postinfluenza Vaccination Idiopathic Thrombocytopenic Purpura in Three Elderly Patients Sun, 21 Feb 2016 11:33:33 +0000 http://www.hindawi.com/journals/crihem/2016/7913092/ The etiologies of secondary idiopathic thrombocytopenic purpura (ITP) include infection, autoimmune disease, and immunodeficiency. We report the cases of three elderly patients who developed ITP after receiving influenza vaccinations. The platelet count of an 81-year-old woman fell to 27,000/μL after she received an influenza vaccination. A 75-year-old woman developed thrombocytopenia (5,000 platelets/μL) after receiving an influenza vaccination. An 87-year-old woman whose laboratory test values included a platelet count of 2,000/μL experienced genital bleeding after receiving an influenza vaccination. After Helicobacter pylori (HP) eradication or corticosteroid treatment, all of the patients’ platelet counts increased. Influenza vaccination is an underlying etiology of ITP in elderly patients. HP eradication or corticosteroid treatment is effective for these patients. Clinicians should be aware of the association between ITP and influenza vaccinations. Joji Nagasaki, Masahiro Manabe, Kentaro Ido, Hiroyoshi Ichihara, Yasutaka Aoyama, Tadanobu Ohta, Yoshio Furukawa, and Atsuko Mugitani Copyright © 2016 Joji Nagasaki et al. All rights reserved. A 54-Year-Old Woman with Donor Cell Origin of Multiple Myeloma after Allogeneic Hematopoietic Stem Cell Transplantation for the Treatment of CML Thu, 18 Feb 2016 11:22:34 +0000 http://www.hindawi.com/journals/crihem/2016/6751914/ Chronic myeloid leukemia is a myeloproliferative disorder that may be treated with hematopoietic stem cell transplantation (HSCT). While posttransplantation relapse of disease resulting from a failure to eradicate the patient’s original leukemia could occur, patients may also rarely develop a secondary malignancy or myelodysplastic syndrome (MDS) of donor origin termed donor cell leukemia (DCL). Cases of donor-derived acute myeloid leukemia (AML) or MDS after HSCT or solid tumor transplantation have been published. However, very few cases of donor-derived multiple myeloma (MM) exist. We describe a patient who developed a donor-derived MM following allogeneic HSCT from a sibling donor. Erika Maestas, Shikha Jain, and Patrick Stiff Copyright © 2016 Erika Maestas et al. All rights reserved. RSV-Related Thrombocytopenia Associated with Transient Cytogenetic Abnormalities in a Recipient of Umbilical Cord Blood Transplantation Tue, 02 Feb 2016 14:17:03 +0000 http://www.hindawi.com/journals/crihem/2016/8628507/ Respiratory syncytial virus (RSV) infections are associated with thrombocytopenia. The underlying mechanism of thrombocytopenia in this setting is unknown. Herein, we report a case of RSV-related thrombocytopenia associated with transient cytogenetic abnormalities that occurred following umbilical cord blood transplantation. Sabita D. Pokhrel, Diane L. Persons, and Omar S. Aljitawi Copyright © 2016 Sabita D. Pokhrel et al. All rights reserved. Acute Lymphoblastic Leukemia Arising in CALR Mutated Essential Thrombocythemia Thu, 21 Jan 2016 13:27:21 +0000 http://www.hindawi.com/journals/crihem/2016/6545861/ The development of acute lymphoblastic leukemia in an existing myeloproliferative neoplasm is rare with historical cases unable to differentiate between concomitant malignancies or leukemic transformation. Molecular studies of coexisting JAK2 V617F-positive myeloproliferative neoplasms and mature B cell malignancies indicate distinct disease entities arising in myeloid and lymphoid committed hematopoietic progenitor cells, respectively. Mutations of CALR in essential thrombocythemia appear to be associated with a distinct phenotype and a lower risk of thrombosis yet their impact on disease progression is less well defined. The as yet undescribed scenario of pro-B cell acute lymphoblastic leukemia arising in CALR mutated essential thrombocythemia is presented. Intensive treatment for the leukemia allowed for expansion of the original CALR mutated clone. Whether CALR mutations in myeloproliferative neoplasms predispose to the acquisition of additional malignancies, particularly lymphoproliferative disorders, is not yet known. Stephen E. Langabeer, Karl Haslam, David O’Brien, Johanna Kelly, Claire Andrews, Ciara Ryan, Richard Flavin, Patrick J. Hayden, and Christopher L. Bacon Copyright © 2016 Stephen E. Langabeer et al. All rights reserved. T Cell/Histiocyte-Rich Large B Cell Lymphoma of the Thymus: A Diagnostic Pitfall Wed, 20 Jan 2016 16:08:01 +0000 http://www.hindawi.com/journals/crihem/2016/2942594/ T cell/histiocyte-rich large B cell lymphoma (THRLBCL) is predominantly a nodal disease, with extranodal involvement, such as bone marrow, spleen, and liver. However, primary THRLBCL has never been reported in the thymus in the English literature. Here we report a case of THRLBCL presenting as mediastinal mass and lymphadenopathy. Based on the frozen section diagnosis of “thymoma,” a 12 cm mass was excised. A year later she developed multiple enlarged lymph nodes and pulmonary nodules. Consultant review of the excised mediastinal mass showed scattered large atypical cells that were CD20+ and PAX-5+ and negative for pan-cytokeratin, AE1, and AE3, compatible with THRLBCL and excluding thymoma. The excised lymph nodes were replaced by diffuse infiltrate of small CD3+ lymphocytes and histiocytes with intermingled large CD20+ B lymphoma cells scattered throughout the section. A diagnosis of THRLBCL was made in lymph node, similar to previous thymic lesion. A clonal rearrangement of immunoglobulin heavy chain (IGH) gene was detected, further supporting the diagnosis. This is the first reported case of THRLBCL in thymus. Correct recognition of this entity is critical, because of the difference in therapeutic impact on these patients. Jie Xu, Xiaojun Wu, and Vishnu Reddy Copyright © 2016 Jie Xu et al. All rights reserved. Higher Risk Myelodysplastic Syndromes in Patients with Well-Controlled HIV Infection: Clinical Features, Treatment, and Outcome Wed, 20 Jan 2016 05:52:57 +0000 http://www.hindawi.com/journals/crihem/2016/8502641/ Introduction. In advanced HIV prior to combination antiretroviral therapy (ART), dysplastic marrow changes occurred and resolved with ART. Few reports of myelodysplastic syndromes (MDS) in well-controlled HIV exist and management is undefined. Methods. Patients with well-controlled HIV and higher risk MDS were identified; characteristics, treatment, and outcomes were reviewed. Results. Of 292 MDS patients since 1996, 1 (0.3%) was HIV-positive. A 56-year-old woman presented with cytopenias. CD4 was 1310 cells/mL and HIV viral load <40 copies/mL. Bone marrow biopsy showed RCMD and karyotype included del(5q) and del(7q); IPSS was intermediate-2 risk. She received azacitidine at 75% dose. Cycle 2, at full dose, was complicated by marrow aplasia and possible AML; she elected palliation. Three additional HIV patients with higher risk MDS, aged 56–64, were identified from the literature. All had deletions involving chromosomes 5 and 7. MDS treatment of 2 was not reported and one received palliation; all died of AML. Conclusion. Four higher risk MDS in well-controlled HIV were below the median age of diagnosis for HIV-negative patients; all had adverse karyotype. This is the first report of an HIV patient receiving MDS treatment with azacitidine. Cytopenias were profound and dosing in HIV patients should be considered with caution. Bradley T. Williamson and Heather A. Leitch Copyright © 2016 Bradley T. Williamson and Heather A. Leitch. All rights reserved. Primary Myelofibrosis Presenting as Extramedullary Hematopoiesis in a Transplanted Liver Graft: Case Report and Review of the Literature Sun, 17 Jan 2016 16:55:05 +0000 http://www.hindawi.com/journals/crihem/2016/9515404/ Primary myelofibrosis (PMF) commonly results in extramedullary hematopoiesis (EMH) in the spleen and liver as well as a variety of other organs. We present a first report of a unique presentation of PMF in a liver transplant recipient patient as EMH in the transplanted liver graft. A 76-year-old man with history of cryptogenic cirrhosis received cadaveric liver transplantation in 1996. He maintained a normal graft function and stable hematologic parameters until 2013 when he presented with anemia and progressive fatigue. Extensive work-up did not identify the etiology of the recent decline in his hemoglobin; thus a liver biopsy was done which showed findings of EMH within the sinusoids with increased megakaryocytes, some with atypical morphology. A BM biopsy revealed a hypercellular marrow, moderately increased reticulin fibrosis, and features consistent with primary myelofibrosis. Abdominal imaging showed a normal-size spleen and did not identify any sites of EMH outside of the liver. The diagnosis of myelofibrosis was thus made, and this case demonstrated predominant tropism to a transplanted liver graft with absence of EMH elsewhere. We would thus like to emphasize that findings of EMH in subjects with no preexisting hematologic neoplasm should warrant close follow-up and assessment. Ghulam Rehman Mohyuddin and Abdulraheem Yacoub Copyright © 2016 Ghulam Rehman Mohyuddin and Abdulraheem Yacoub. All rights reserved. Massive Retroperitoneal Hemorrhage as an Initial Presentation of a Rare and Aggressive Form of Multiple Myeloma Thu, 14 Jan 2016 14:11:35 +0000 http://www.hindawi.com/journals/crihem/2016/8206826/ Multiple myeloma, a plasma cell neoplasm, presents most commonly with anemia, hypercalcemia, renal failure, and bone pain. Only few cases of clinical aggressive presentation associated with bleeding were reported in the medical literature. The reported cases included gastrointestinal bleeding and cardiac tamponade. Spontaneous retroperitoneal haemorrhage as initial presentation has not been so far reported. We hereby report a case of a 64-year-old female who was found to have catastrophic hemorrhage in the retroperitoneal region that extended into intrathecal space causing cord compression. The case posed a significant diagnostic and management dilemma. This case emphasizes the need to think broadly and include multiple myeloma in the diagnosis of unexplained massive retroperitoneal bleeding. Aydah Alawadhi and Laszlo Leb Copyright © 2016 Aydah Alawadhi and Laszlo Leb. All rights reserved. Light Chain Escape in 3 Cases: Evidence of Intraclonal Heterogeneity in Multiple Myeloma from a Single Institution in Poland Thu, 31 Dec 2015 09:24:47 +0000 http://www.hindawi.com/journals/crihem/2015/809840/ We report three cases of light chain escape (LCE) at a single institution in Poland, including an interesting case of biclonal monoclonal gammopathy of undetermined significance (MGUS) that satisfied the criteria for progression to light chain multiple myeloma (LCMM) with a rapid rise in serum free light chain (FLC) levels, following steroidal treatment for simultaneous temporal artery inflammation and polymyalgia rheumatica (PMR). In the three cases discussed, progression of the disease by light chain escape was associated with rapid and severe renal impairment, highlighting the necessity for prompt detection of such free light chain-only producing clones in order to prevent the possible development of irreversible end-organ damage. Interestingly, monitoring of these three patients by serum free light chain assay (sFLC) and retrospective heavy/light chain analysis (HLC) detected this clonal evolution prior to clinical relapse and suggests that these assays represent important additional tools for more accurate monitoring of multiple myeloma patients. Maria Kraj, Barbara Kruk, Kelly Endean, Krzysztof Warzocha, Katarzyna Budziszewska, and Monika Dąbrowska Copyright © 2015 Maria Kraj et al. All rights reserved. Donor Cell Myeloid Sarcoma in an Umbilical Cord Transplant Patient: A Case Report and a Review of the Literature Mon, 28 Dec 2015 07:22:25 +0000 http://www.hindawi.com/journals/crihem/2015/186869/ Donor cell leukemia (DCL) represents a rare complication of allogeneic transplantation. The precise incidence remains unclear, though it may be higher following umbilical cord blood transplants. Here, we present an unusual case of a patient with B-ALL who presented with a donor derived myeloid sarcoma of the heart following a double cord blood transplant. To our knowledge, it is the first case of sarcomatous or chloromatous presentation of DCL following a UCBT. Christi Hayes, Bruce Petersen, and Adriana Malone Copyright © 2015 Christi Hayes et al. All rights reserved. Acute Unilateral Blindness from Superior Ophthalmic Vein Thrombosis: A Rare Presentation of Nephrotic Syndrome from Class IV Lupus Nephritis in the Absence of Antiphospholipid or Anticardiolipin Syndrome Mon, 21 Dec 2015 14:10:45 +0000 http://www.hindawi.com/journals/crihem/2015/413975/ Patients with systemic lupus erythematosus (SLE) are at high risk of arterial and venous thrombosis secondary to anti-phospholipid antibodies. Herein, we are presenting an interesting case of venous thrombosis in a patient with SLE in the absence of anti-phospholipid antibodies. Firas Baidoun, Rommy Issa, Robert Ali, and Bashar Al-Turk Copyright © 2015 Firas Baidoun et al. All rights reserved. Translocation (6;15)(q12;q15): A Novel Mutation in a Patient with Therapy-Related Myelodysplastic Syndrome Mon, 21 Dec 2015 11:35:48 +0000 http://www.hindawi.com/journals/crihem/2015/318545/ Most myelodysplastic syndromes (MDS) present with loss or gain of chromosomal material and less commonly show translocations as a sole abnormality. In addition, certain translocations are more commonly seen in MDS than others, but to our knowledge, the presence of t(6;15) has not been reported in MDS, specifically therapy-related MDS (t-MDS) cases. Patients with t-MDS, a group of heterogeneous stem cell related disorders resulting as a latent complication of cytotoxic and/or radiation therapy, generally tend to have a poorer prognosis than de novo MDS. We present a unique case of a patient who initially presented with acute myeloid leukemia (AML) with a normal karyotype and FLT3-ITD and NPM1 mutations. The patient was successfully treated with chemotherapy and an autologous bone marrow transplant but subsequently developed a new FLT3-ITD negative t-MDS with a unique translocation, t(6;15)(q12;q15), three years after transplant. To our knowledge, this unique sole translocation has never been reported in MDS or t-MDS and given her successful response to treatment and remission, presence of this translocation may have some prognostic value. Saba F. Ali, Rebecca J. Sonu, Denis M. Dwyre, Brian A. Jonas, and Hooman H. Rashidi Copyright © 2015 Saba F. Ali et al. All rights reserved. Pulmonary Complications of Azanucleoside Therapy in Patients with Myelodysplastic Syndrome and Acute Myelogenous Leukemia Sun, 20 Dec 2015 14:28:28 +0000 http://www.hindawi.com/journals/crihem/2015/357461/ Our primary aim was to identify potential risk factors and clinical outcome of azanucleoside induced pulmonary complications in patients with myelodysplastic syndrome (MDS) and Acute Myelogenous Leukemia (AML). We present an 89-year-old female with MDS derived AML who developed fatigability, hypoxemia, and bilateral lung infiltrates indicating interstitial lung disease after 11 cycles of azanucleoside. In addition, we describe a cohort of six MDS patients with fever, cough, dyspnea, and pulmonary infiltrates at early time point during azanucleoside treatment. Early and late onset of pulmonary manifestations suggest different pathogenic mechanisms. Brief azanucleoside discontinuation and steroids led to rapid improvement in symptoms. Manuel Molina, Sarvari Yellapragada, Martha Mims, Effie Rahman, and Gustavo Rivero Copyright © 2015 Manuel Molina et al. All rights reserved. Hairy Cell Leukemia Presenting with Isolated Skeletal Involvement Successfully Treated by Radiation Therapy and Cladribine: A Case Report and Review of the Literature Wed, 16 Dec 2015 11:29:18 +0000 http://www.hindawi.com/journals/crihem/2015/803921/ We describe an unusual case of hairy cell leukemia (HCL) in a 55-year-old male presenting with isolated skeletal disease as the initial manifestation without abnormal peripheral blood counts, bone marrow involvement, or splenomegaly. To the best of our knowledge, there have been only two previous reports of a similar case. The patient presented with pain in the right femur. Anteroposterior radiographs of both femurs revealed mixed lytic-sclerotic lesions. PET scan showed multiple metastatic lesions on axial skeleton, pelvis, and both femurs. Histopathological examination of the bone biopsy revealed an infiltrate of HCL. Localized radiation therapy to both proximal femurs and subsequently 4 weeks later, a 7-day course of 0.1 mg/kg/day cladribine provided complete remission with relief of symptoms and resolution of bone lesions. We addressed the manifestations and management of HCL patients with skeletal involvement. Ipek Yonal-Hindilerden, Fehmi Hindilerden, Sanem Bulut-Dereli, Eren Yıldız, Ibrahim Oner Dogan, and Meliha Nalcaci Copyright © 2015 Ipek Yonal-Hindilerden et al. All rights reserved. Aberrant “Barbed-Wire” Nuclear Projections of Neutrophils in Trisomy 18 (Edwards Syndrome) Sun, 06 Dec 2015 05:48:02 +0000 http://www.hindawi.com/journals/crihem/2015/163857/ We discuss the significance of neutrophils with increased, aberrant nuclear projections mimicking “barbed-wire” in a newborn child with trisomy 18 (T18). Increased, aberrant nuclear projections have been previously reported in trisomy of the D group of chromosomes (chromosomes 13, 14, and 15), and we report similar findings in a patient with T18. The peripheral blood smear showed relative neutrophilia with the majority (37%) of neutrophils showing two or more thin, rod-shaped or spike-shaped, and often pedunculated aberrant nuclear projections. The number of projections ranged from 2 to 6 per cell, averaged 2 per affected neutrophil, and ranged in length from 0.22 μm to 0.83 μm. This case confirms that the morphologic finding described is not restricted to trisomy of one of the chromosomes in group D, as implied in the literature. Basil M. Kahwash, Nicholas B. Nowacki, and Samir B. Kahwash Copyright © 2015 Basil M. Kahwash et al. All rights reserved.