Case Reports in Hematology The latest articles from Hindawi Publishing Corporation © 2014 , Hindawi Publishing Corporation . All rights reserved. Mantle Cell Lymphoma Mimicking Rectal Carcinoma Thu, 17 Apr 2014 00:00:00 +0000 Mantle cell lymphoma (MCL) is a mature B-cell non-Hodgkin lymphoma. After the (11;14) translocation was identified as its constant finding in 1992, MCL was recognized as a separate subgroup of non-Hodgkin lymphoma (NHL). In MCL, extranodal involvement may be observed in the bone marrow, the spleen, the liver, and the gastrointestinal system (GIS). Cases of MCL that present with a massive and solitary rectal mass are rare in the literature. In this case report, our aim was to present an MCL patient with a rarely observed solitary rectal involvement mimicking rectal carcinoma and to discuss treatment options for this patient. Engin Kelkitli, Hilmi Atay, Levent Yıldız, Ahmet Bektaş, and Mehmet Turgut Copyright © 2014 Engin Kelkitli et al. All rights reserved. Donor Cell Myeloid Sarcoma Mon, 14 Apr 2014 09:07:29 +0000 Donor cell derived malignancies are a rare and interesting complication of allogeneic bone marrow transplantation. We present a case of a 56-year-old male with donor cell myeloid sarcoma of the stomach and myocardium. Mark A. Walshauser, Aileen Go, Payal Sojitra, Girish Venkataraman, and Patrick Stiff Copyright © 2014 Mark A. Walshauser et al. All rights reserved. T-Cell Lymphoblastic Leukemia/Lymphoma: Relapse 16 Years after First Remission Mon, 14 Apr 2014 00:00:00 +0000 Little information is available regarding late relapse in patients with T-lymphoblastic leukemia/lymphoma (T-LBL). Because of the aggressive nature of this disease, relapse is common and often happens early. Late relapses are rare and generally occur within a few years after initial remission. The relapse rate after 3 years has been reported to steadily decrease over time yet does not parallel with cure. We report a case of a 26-year-old female with T-LBL and relapse 16 years after her first remission with successful treatment with HyperCVAD and L-asparaginase. Lauren Elreda, Manpreet Sandhu, Xinlai Sun, Wondwessen Bekele, Alice J. Cohen, and Maya Shah Copyright © 2014 Lauren Elreda et al. All rights reserved. Spontaneous Intestinal Perforation: An Atypical Presentation of Neutropenic Enterocolitis—A Case Report Thu, 06 Mar 2014 00:00:00 +0000 Background. Neutropenic enterocolitis is one of the most common gastrointestinal complications seen in patients who are receiving chemotherapy for leukemia. Severe neutropenia is the main underlying factor of this pathology. It is characterized by fever and abdominal pain. Case Presentation. Herein, we report a case of neutropenic enterocolitis which presented with intestinal perforation in an afebrile patient who was diagnosed with acute lymphoblastic leukemia and was receiving induction chemotherapy. Conclusion. We aimed to emphasize the importance of enterocolitis and increase awareness against such severe complications which could have unexpected presentations. Aylin Canbolat Ayhan, Cetin Timur, Ersin Bocu, and Neslihan Gulcin Copyright © 2014 Aylin Canbolat Ayhan et al. All rights reserved. Open Questions in the Management of Nodular Lymphocyte Predominant Hodgkin Lymphoma Thu, 20 Feb 2014 08:18:56 +0000 Localized Nodular Lymphocyte Predominant Hodgkin Lymphoma is a rare disease with an overall good prognosis but frequent late relapses. Due to it’s rarity there is no standard therapeutic approach and pathological diagnosis may be hard. In this paper we discuss the technical aspects of the radiation therapy and histological issues. The new fields reductions proposed for classical Hodgkin lymphoma cannot be applied to early stages Nodular Lymphocyte Predominant Hodgkin lymphomas which are usually treated with radiation therapy without systemic chemotherapy. Marguerite Tyran, Laurence Gonzague, Reda Bouabdallah, and Michel Resbeut Copyright © 2014 Marguerite Tyran et al. All rights reserved. Hermansky-Pudlak Syndrome: A Case Report Thu, 20 Feb 2014 07:21:21 +0000 Objective. The aim of this paper is to report the case of a patient diagnosed with Hermansky-Pudlak syndrome, as a result of bleeding diathesis. Clinical Presentation and Intervention. A 23-year-old male presented with recurrent epistaxis and, upon physical examination, was found to be remarkable for albinism and suborbital ecchymosis. The absence of dense bodies in the platelets was demonstrated using electron microscopy. This patient was (slowly) administered one unit of a platelet suspension, and his bleeding decreased considerably. Conclusion. This case shows that Hermansky-Pudlak syndrome should be considered in the differential diagnosis of a patient presenting with bleeding diathesis, when the clinical presentation also includes oculocutaneous albinism and visual problems. Ilhami Berber, Mehmet Ali Erkurt, Irfan Kuku, Emin Kaya, Mustafa Koroglu, Ilknur Nizam, Mehmet Gul, and Recep Bentli Copyright © 2014 Ilhami Berber et al. All rights reserved. Lenalidomide and Dexamethasone for a Patient of POEMS Syndrome Presenting with Massive Ascites Wed, 19 Feb 2014 12:45:29 +0000 POEMS syndrome is a multisystem disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. POEMS syndrome is a rare cause of refractory ascites. We report the case of a patient with POEMS syndrome presenting with massive ascites who was treated with very-low-dose lenalidomide and dexamethasone. A 57-year-old Japanese man was admitted to our hospital with pleural effusion, massive ascites, and leg edema. The diagnosis of POEMS syndrome was made based on the combination of the following findings: peripheral neuropathy, organomegaly, endocrinopathy, serum monoclonal protein elevation, skin changes, plasma VEGF elevation, and evidence of extravascular volume overload. Renal dysfunction induced by biopsy-proven renal involvement of POEMS syndrome was observed. Massive ascites of the patient dramatically diminished with long-time treatment of very-low-dose lenalidomide and dexamethasone. Lenalidomide seems to be a very promising therapy for POEMS syndrome presenting with extravascular volume overload such as edema, pleural effusion, and ascites. Very-low-dose lenalidomide might be effective especially for the patients with POEMS-related nephropathy. Shuji Ueda, Sayoko Yonemoto, Kazumasa Oka, Naohiko Fujii, Keiichi Nakata, Hitomi Matsunaga, Seiko Kataoka, Yuki Iwama, Hiroyuki Narahara, Yuichi Yasunaga, Yoshiaki Inui, and Sumio Kawata Copyright © 2014 Shuji Ueda et al. All rights reserved. Pancytopenia in a 70-Year-Old African-American Male: An Unusual Presentation of a Rare Disease Sun, 16 Feb 2014 14:19:00 +0000 Hairy cell leukemia is a rare lymphoid neoplasm arising from mature B-lymphocytes. Clinically, the disease presents with splenomegaly and abdominal discomfort, frequent infections, fatigue and bleeding because of related cytopenias. Bone marrow biopsy is essential for diagnosis. Below we describe a case of a 70-year-old African-American male who presented to our hematology clinic complaining of fatigue. Clinical exam and computed tomography imaging did not reveal splenic enlargement. Blood work-up revealed pancytopenia and bone marrow was diagnostic for hairy cell leukemia.The patient was started on cladribine, with gradual improvement of his symptoms and blood count abnormalities. Therefore, it is essential to keep hairy cell leukemia in the differential of pancytopenia even in the absence of a splenomegaly. Aibek E. Mirrakhimov, Alaa M. Ali, Aram Barbaryan, Nwabundo Anusim, Raya Saba, Shawn G. Kwatra, Nasir Hussain, Teresita Zdunek, and Alan D. Gilman Copyright © 2014 Aibek E. Mirrakhimov et al. All rights reserved. Chlorambucil-Induced Acute Interstitial Pneumonitis Wed, 12 Feb 2014 12:52:14 +0000 Chlorambucil is an alkylating agent commonly used in treatment of chronic lymphocytic leukemia (CLL). We report a case of interstitial pneumonitis developing in an 83-year-old man 1.5 months after completing a six-month course of chlorambucil for CLL. The interstitial pneumonitis responded to therapy with prednisone. We performed a systematic review of literature and identified 13 other case reports of chlorambucil-induced pulmonary toxicity, particularly interstitial pneumonitis. No unifying risk factor could be discerned and the mechanism of injury remains unknown. In contrast, major randomized trials of chlorambucil therapy in CLL have not reported interstitial pneumonitis as an adverse effect, which may be due to the rarity of the phenomenon or due to underreporting of events occurring after completion of treatment. Clinicians should consider drug-induced interstitial pneumonitis in the differential diagnosis of a suggestive syndrome developing even after discontinuation of chlorambucil. Hammad Shafqat and Adam J. Olszewski Copyright © 2014 Hammad Shafqat and Adam J. Olszewski. All rights reserved. Epstein-Barr Virus Infection in an Elderly Nonimmunocompromised Adult Successfully Treated with Rituximab Mon, 10 Feb 2014 06:54:44 +0000 Epstein-Barr virus (EBV) is a ubiquitous virus that commonly affects children and adolescents. In addition to causing a viral illness, it is also associated with various malignancies in particular B cell lymphomas and lymphoproliferative disorders. Differentiating between the two processes can be a diagnostic challenge. Here, we present a case of an atypical EBV infection in an elderly patient with severe systemic symptoms, multiorgan involvement, lymphadenopathy, and negative EBV serology. Excisional lymph node biopsy demonstrated features of a lymphoproliferative process involving EBV. Despite supportive care, she experienced continued clinical deterioration and was successfully treated with rituximab. This case illustrates the diagnostic challenges of these cases particularly in the elderly who may have age related immunosenescence, the utility of EBV PCR testing, and the clinical efficacy of rituximab in clearing the infected cells. Jacob P. Smeltzer, Matthew T. Howard, Wilson I. Gonsalves, and Thomas E. Witzig Copyright © 2014 Jacob P. Smeltzer et al. All rights reserved. Successful Outcome of Chronic Intrahepatic Cholestasis in an Adult Patient with Sickle Cell/β+ Thalassemia Sun, 09 Feb 2014 12:15:20 +0000 Sickle cell/ thalassemia (Hb S/thal) is considered as a variant form of sickle cell disease. Acute episodes of vasoocclusive pain crisis are characteristic for sickle cell disorders and may be complicated by an acute or chronic life-threatening organ dysfunction. Chronic intrahepatic cholestasis is a rare and severe complication in sickle cell disease, characterized by marked hyperbilirubinemia and acute hepatic failure with an often fatal course. Despite the fact that patients with Hb S/thal usually have a mild type of disease, herein we describe an interesting case of chronic intrahepatic cholestasis with successful outcome in an adult patient with Hb S/thal. Efthymia Vlachaki, Panagiotis Andreadis, Nikolaos Neokleous, Aleka Agapidou, Evaggelia Vetsiou, Panagiotis Katsinelos, and Panagiota Boura Copyright © 2014 Efthymia Vlachaki et al. All rights reserved. Prolonged Survival of a Refractory Acute Myeloid Leukemia Patient after a Third Hematopoietic Stem Cell Transplantation with Umbilical Cord Blood following a Second Relapse Sun, 09 Feb 2014 07:33:32 +0000 Although hematopoietic stem cell transplantation (HSCT) has been considered to be the only way for potential cure of relapsed acute myeloid leukemia (AML), there has been no report on a third HSCT in patients with multiple relapsed AML. Here, we report a case of 53-year-old female who received a successful third allogeneic HSCT after relapse of AML following a second allogeneic HSCT. She was treated with a toxicity reduced conditioning regimen and received direct intrabone cord blood transplantation (CBT) using a single unit of 5/6 HLA-matched cord blood as a graft source. Graft-versus-host disease prophylaxis was performed with a single agent of tacrolimus to increase graft-versus-leukemia effect. She is in remission for 8 months since the direct intrabone CBT. This report highlights not only the importance of individually adjusted approach but also the need for further investigation on the role of HSCT as a treatment modality in patients with refractory or multiple relapsed AML. Suk-young Lee, Naoki Kurita, Koichiro Maie, Masanori Seki, Yasuhisa Yokoyama, Kazumi Suzukawa, Yuichi Hasegawa, and Shigeru Chiba Copyright © 2014 Suk-young Lee et al. All rights reserved. “Almost Bleeding to Death”: The Conundrum of Acquired Amegakaryocytic Thrombocytopenia Thu, 06 Feb 2014 09:41:07 +0000 Acquired amegakaryocytic thrombocytopenia (AAT) is a rare hematological disorder causing severe thrombocytopenia and bleeding. Previous in vitro studies postulated both cell-mediated suppression of megakaryocytopoiesis in early megakaryocytic progenitor cells and humoral-mediated suppression by anti-thrombopoietin antibodies as possible etiologies of AAT. Patients with AAT usually present with severe bleeding and thrombocytopenia that is unresponsive to steroids and intravenous immunoglobulin (IVIG). Although standard guidelines have not been established for management of AAT, a few case reports have indicated a response to immunosuppressive treatment. The prompt recognition of this disease entity is essential in view of the substantial risk of morbidity and mortality from excessive bleeding. We report a case of AAT successfully treated with equine antithymocyte globulin (ATG) and cyclosporine (CSP). Gabrielle Elena Brown, Hani M. Babiker, Carlos L. Cantu, Andrew M. Yeager, and Ravitharan Krishnadasan Copyright © 2014 Gabrielle Elena Brown et al. All rights reserved. The Enigmatic Kikuchi-Fujimoto Disease: A Case Report and Review Thu, 06 Feb 2014 07:57:41 +0000 We report this case of a 33-year-old African American woman who presented to the clinic with preauricular and submandibular masses that she had noticed 6 weeks earlier. She gave a remote history of noticing bilateral cervical masses 3 years prior to this presentation that had not been investigated at the time and resolved spontaneously. Excisional biopsies of the cervical lymph nodes showed morphologic and immunophenotypic findings suggestive of Kikuchi Fujimoto disease (KFD). KFD is an uncommon, self-limited, and perhaps an underdiagnosed entity with an excellent prognosis. It mimics malignant lymphoma in presentation and therefore an accurate clinicopathological differentiation is crucial. Hassan Tariq, Vinaya Gaduputi, Arsalan Rafiq, and Roopalekha Shenoy Copyright © 2014 Hassan Tariq et al. All rights reserved. Recurrent and Progressive Abdominal Pain and Enteritis in a Japanese Patient with Paroxysmal Nocturnal Hemoglobinuria Mon, 27 Jan 2014 09:54:20 +0000 This case report describes a young male patient with recurrent abdominal pain persisting for more than 16 months. Clinical investigations showed signs of inflammation and pancytopenia. A diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) was made 9 months after the onset of the abdominal pain, following endoscopic examinations that revealed evidence of a previously unknown hemorrhage. Regular monitoring indicated that the abdominal pain was associated with elevations in lactate dehydrogenase, C-reactive proteins, and D-dimer levels. The patient started treatment with the complement inhibitor eculizumab shortly after it was approved for use in Japanese PNH patients with hemolysis. Resolution of the abdominal pain and normalization of clinical parameters were noted within 3 weeks from treatment initiation. Akihisa Hino, Yukiko Yamashita, Mitsuhiro Yamaguchi, and Yasuhiko Azenishi Copyright © 2014 Akihisa Hino et al. All rights reserved. Secondary Hemophagocytic Syndrome Associated with Richter’s Transformation in Chronic Lymphocytic Leukemia Thu, 16 Jan 2014 13:36:42 +0000 Hemophagocytic syndrome (HPS) is an extremely rare condition arising from the overactivation of one’s own immune system. It results in excessive inflammation and tissue destruction. Prompt initiation of treatment is warranted in either scenario in order to decrease mortality. Most cases are triggered by infectious agents, malignancy, or drugs. We describe the first case of a CLL patient presenting with HPS due to acquisition of EBV-related large cell lymphoma in the setting of profound immunodeficiency. Nura El-Haj, Wilson I. Gonsalves, Vinay Gupta, Jacob P. Smeltzer, Sameer A. Parikh, Preet P. Singh, and Naseema Gangat Copyright © 2014 Nura El-Haj et al. All rights reserved. Lung Adenocarcinoma in a Patient with Plasmacytoma Sun, 22 Dec 2013 11:58:18 +0000 An increased risk of second malignancy is well recognized in patients treated for plasma cell neoplasms. However, second solid tumor is very rare in such patients. We report a case of a 68-year-old woman with plasmacytoma who developed lung adenocarcinoma. Atsunori Hiasa, Kazunori Nakase, Kazuo Fukutome, Hideki Nomura, Setsuko Ueno, Toshiro Mizuno, Naoyuki Katayama, and Toshiaki Takeuchi Copyright © 2013 Atsunori Hiasa et al. All rights reserved. A Case of an Acquired Factor VIII Inhibitor Complicated by Multiple Treatment-Related Opportunistic Infections and Review of the Literature Wed, 18 Dec 2013 10:48:24 +0000 This case report describes a patient with an idiopathic acquired Factor VIII inhibitor and severe bleeding. She was treated with rituximab after failing first-line treatment with steroids and cyclophosphamide. Two months following rituximab treatment, our patient developed a succession of severe opportunistic infections requiring intensive care unit admission. Over a period of 12 weeks she required treatment for Pseudomonas aeruginosa septicaemia, herpes simplex gingivostomatitis and pharyngotonsillitis, clostridium difficile-related diarrhoea, systemic cytomegalovirus infection, pneumocystis jiroveci, and invasive pulmonary aspergillosis lung infections. After significant rehabilitation, the patient was finally discharged following a 5-month admission. This case highlights the complexity of balancing a life-threatening condition with the side effects of treatment. It also raises the issue of routine prophylaxis for immunosuppression in nonmalignant conditions, which will become a common dilemma with the expanding indications for rituximab use. Anna L. Hutchinson, Yi Ling Tan, and Giselle Kidson-Gerber Copyright © 2013 Anna L. Hutchinson et al. All rights reserved. Malignant Phyllodes Tumor and Acute Megakaryoblastic Leukemia Sharing a Common Clonal Origin Tue, 17 Dec 2013 14:23:40 +0000 There is a well-known association in male patients between mediastinal germ cell tumors (GCT) and hematologic malignancies, with a propensity towards acute megakaryoblastic leukemia. These rare malignancies have been shown to share a common clonal origin, often deduced from the finding of isochromosome 12p, i(12p), in cells from both the solid tumor and the leukemia, and thus are now known to represent different manifestations of the same clonal process. We treated a young female patient with a malignant phyllodes tumor followed by an acute megakaryoblastic leukemia and found several of the same marker chromosomes by karyotype analysis of cells from both the tumor and the leukemia implying a common clonal origin of the two. To the best of our knowledge, this has not been demonstrated in phyllodes tumors before, but indicates that the same type of leukemization may occur of this tumor as has been described in mediastinal GCT. Yngvar Fløisand, Klaus Beiske, Geir Erland Tjønnfjord, Dag Heldal, Bodil Bjerkehagen, Mona-Elisabeth Revheim, Sverre Heim, Øyvind Sverre Bruland, Kirsten Sundby Hall, Anne Tierens, and Jan Delabie Copyright © 2013 Yngvar Fløisand et al. All rights reserved. Hemophagocytic Lymphohistiocytosis Complicating Erythroleukemia in a Child with Monosomy 7: A Case Report and Review of the Literature Thu, 12 Dec 2013 11:36:05 +0000 Herein, the first case of childhood erythrophagocytosis following chemotherapy for erythroleukemia in a child with monosomy 7 is reported. A 5-year-old boy presented with anemia, thrombocytopenia, and hepatosplenomegaly in whom erythroleukemia was diagnosed. Prolonged pancytopenia accompanied by persistent fever and huge splenomegaly and hepatomegaly became evident after 2 courses of chemotherapy. On bone marrow aspiration, macrophages phagocytosing erythroid precursors were observed and the diagnosis of HLH was established; additionally, monosomy 7 was detected on bone marrow cytogenetic examination. In conclusion, monosomy 7 can lead to erythrophagocytosis associated with erythroid leukemia and should be considered among the chromosomal abnormalities contributing to the association. Samin Alavi, Maryam Ebadi, Alireza Jenabzadeh, M. T. Arzanian, and Sh. Shamsian Copyright © 2013 Samin Alavi et al. All rights reserved. Safety of Pegfilgrastim (Neulasta) in Patients with Sickle Cell Trait/Anemia Wed, 11 Dec 2013 14:30:49 +0000 Pegfilgrastim (Neulasta) is a recombinant filgrastim (human granulocyte colony-stimulating factor (G-CSF)) attached to a polyethylene glycol (PEG) molecule and is given as part of chemotherapy regimens that are associated with significant myelosuppression and risk for febrile neutropenia. Prescribing information available on manufacturer’s website for the drug warns us about possible severe sickle cell crises related to the medication but does not report the actual incidence or the use in patients with sickle cell trait. Caution is advised when using it in patients with sickle cell disease. Here we present a case of a Caucasian female with known sickle cell trait (SCT) with no prior complications who developed a presumed sickle cell crisis after getting Neulasta, as a part of the chemotherapy regimen used to treat her breast cancer. Based on our literature review, this appears to be the first case report of a patient with SCT developing a sickle cell crisis with the pegylated form of recombinant filgrastim. Given the dearth of literature regarding the use of G-CSF and its related pegylated forms in patients with sickle cell anemia and sickle cell trait, a discussion of potential mechanisms and review of current literature and guidelines is also presented. Pashtoon Murtaza Kasi, Mrinal M. Patnaik, and Prema P. Peethambaram Copyright © 2013 Pashtoon Murtaza Kasi et al. All rights reserved. Blastic Plasmacytoid Dendritic Cell Leukemia Successfully Treated by Autologous Hematopoietic Stem Cell Transplantation to a Remission of 48-Month Duration Wed, 11 Dec 2013 10:39:03 +0000 Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare subtype of AML characterized by the clonal proliferation of precursors of plasmacytoid dendritic cells. It presents with an aggressive behavior. The clinical findings include cytopenia, particularly thrombocytopenia. Although it responds well to chemotherapy initially, the relapse is a rule and prognosis is very poor. There is limited data published in the literature, making it very problematic to define the biological and clinical features, hence, the appropriate therapeutic approach. There are various treatment methods such as multiagent chemotherapy based on ALL or AML and/or hematopoietic stem cell transplantation. However, none of them is approved as a standard therapy. From this point of view, we herein report a 20-year-old case at onset of a leukemic form of BPDCN who survived 48 months after autologous hematopoietic stem cell transplantation. Deniz Goren Sahin, Olga Meltem Akay, Hava Üsküdar Teke, Neslihan Andıc, Eren Gunduz, and Zafer Gulbas Copyright © 2013 Deniz Goren Sahin et al. All rights reserved. More Common Than You Think: Common Variable Immune Deficiency Thu, 05 Dec 2013 11:15:50 +0000 We report a challenging case of a 16-year-old male who presented with thrombocytopenia and eluded a definitive diagnosis for over 2 years. He was initially diagnosed with a viral illness, although he later developed adenopathy and splenomegaly. An evaluation by an oncologist was unrevealing. He worked on a farm with livestock exposure and was later diagnosed with an atypical, zoonotic infection. Despite appropriate antibiotic therapy, the thrombocytopenia and splenomegaly persisted. Further evaluation revealed that he has a relatively common immunologic disorder. He is currently doing well on appropriate therapy for this disorder. Abimbola Aderinto, Vibav Mouli, Danielle F. Resar, and Linda M. S. Resar Copyright © 2013 Abimbola Aderinto et al. All rights reserved. Permanent Sensorineural Deafness in a Patient with Chronic Myelogenous Leukemia Secondary to Intracranial Hemorrhage Tue, 03 Dec 2013 13:06:10 +0000 A 52-year-old male presented with tinnitus and fullness in left ear for one day. Workup revealed a white blood cell count of /L with marked increase in granulocyte series and myeloid precursors on peripheral smear. The initial impression was chronic myelogenous leukemia with hyperleukocytosis, and patient was started on hydration, hydroxyurea, and allopurinol. Patient tolerated bone marrow biopsy well but continued to bleed excessively from the biopsy site. Results confirmed Philadelphia chromosome positive chronic myelogenous leukemia (chronic phase). On day three of hospitalization, patient developed sudden slurred speech along with shaking movements involving extremities. Magnetic resonance imaging revealed multiple hemorrhages throughout the brain. Hydroxyurea was continued until insurance coverage for nilotinib was getting approved. On day nine of hospitalization, patient developed sudden bilateral sensorineural deafness. Repeat magnetic resonance imaging revealed multiple new hemorrhages throughout the brain. Computer tomography of the temporal bones showed inflammatory changes in right and left mastoid cells. Nilotinib was started on day eleven of hospitalization. Patient’s white blood cell count continued to decrease, but there was no improvement in hearing. Four months later, patient was treated with bilateral transmastoid cochlear implants. This case highlights permanent deafness as a hemorrhagic complication secondary to chronic myelogenous leukemia. Sakshi Kapur, Michael Wax, Levin Miles, and Adnan Hussain Copyright © 2013 Sakshi Kapur et al. All rights reserved. Bulky Pulmonary Mucosa-Associated Lymphoid Tissue Lymphoma Treated with Yttrium-90 Ibritumomab Tiuxetan Tue, 26 Nov 2013 16:19:05 +0000 An 84-year-old woman was admitted to our hospital with nonproductive cough and dyspnea on exertion. Computed tomography (CT) scan revealed extensive consolidation in the right lung. She was diagnosed with pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma using CT-guided lung biopsy. Her pulmonary images and respiratory symptoms did not improve two months after receiving 4 cycles of rituximab weekly; therefore, yttrium-90 ibritumomab tiuxetan was chosen as salvage therapy. The abnormal shadow on her pulmonary images was significantly reduced two months later, and she had no symptoms without nonhematological toxicities. She has had no progression for 18 months. Furthermore, radiation pneumonitis has not also been observed. We herein reported bulky pulmonary MALT lymphoma treated with yttrium-90 ibritumomab tiuxetan. Shinobu Tamura, Tokuji Ikeda, Toshio Kurihara, Yoshiteru Kakuno, Hideki Nasu, Yoshio Nakano, Koichi Oshima, and Tokuzo Fujimoto Copyright © 2013 Shinobu Tamura et al. All rights reserved. Biclonal IgD and IgM Plasma Cell Myeloma: A Report of Two Cases and a Literature Review Mon, 18 Nov 2013 13:43:11 +0000 Biclonal plasma cell myelomas producing two different isotypes of immunoglobulins are extremely rare entities; to date, the combination of IgD and IgM secretion by a biclonal plasma cell myeloma has not been reported. Bone marrow biopsy immunohistochemical studies in two cases revealed neoplastic plasma cells coexpressing IgD and IgM, but serum protein electrophoresis identified only the IgM monoclonal paraprotein in both cases. Biclonal plasma cell myelomas, while currently not well characterized in terms of their clinical behavior, should be distinguished from B-cell lymphoma with plasmacytic differentiation, given the different therapeutic implications. Both cases reported herein demonstrated chemotherapy-resistant clinical courses. Zhongchuan W. Chen, Ioanna Kotsikogianni, Jay S. Raval, Christine G. Roth, and Marian A. Rollins-Raval Copyright © 2013 Zhongchuan W. Chen et al. All rights reserved. A Case of Erythrocytosis in a Patient Treated with an Aromatase Inhibitor for Breast Cancer Thu, 07 Nov 2013 11:21:16 +0000 A previously healthy 79-year-old female was referred to hematology for further evaluation of erythrocytosis. Two years earlier she had been diagnosed with ER/PR-positive ductal carcinoma of the breast and was receiving hormonal therapy with exemestane. No secondary cause of erythrocytosis was identified. Serum erythropoietin (EPO) level was normal, and molecular testing for the JAK2 V617F and exon 12 mutations was negative. A bone marrow biopsy showed a mild increase in erythropoiesis, and no spontaneous erythroid colonies were demonstrated. Erythrocytosis is common reason for referral to a hematologist. The myeloproliferative disorder, polycythemia vera, and the rare congenital polycythemias represent primary erythrocytosis. Common secondary causes include smoking, obstructive sleep apnea, and other pulmonary diseases. Erythrocytosis is well described with certain classes of drugs, including androgens. We hypothesize that exemestane contributed to the development of erythrocytosis in our patient. To our knowledge, erythrocytosis has not been previously described in association with aromatase inhibitors. These drugs prevent the conversion of androstenedione and testosterone to estrogen; thus the physiologic mechanisms may be similar to those responsible for erythrocytosis seen with exogenous androgens. These mechanisms are not well understood, but may include altered iron metabolism by a reduction in hepcidin levels. Abhinav Iyengar and Dawn Sheppard Copyright © 2013 Abhinav Iyengar and Dawn Sheppard. All rights reserved. Lupus Nephritis in a Patient with Sickle Cell Disease Thu, 07 Nov 2013 09:13:25 +0000 Introduction. The diagnosis of systemic lupus erythematosus (SLE) in patients with sickle cell disease (SCD) can be difficult to establish because the musculoskeletal, central nervous system, and renal manifestations are similar in both diseases. In the presented case, we highlight the diagnostic challenge that can evolve in patients with a concurrence of both diseases and we establish the importance of early recognition and treatment of lupus nephritis in patients with SCD. Case Presentation. We present a case of a 31-year-old African American female with sickle-C disease (hemoglobin SC) who was admitted to our hospital with complaints of periumbilical abdominal pain associated with intractable nausea and vomiting, abdominal distension, and worsening lower extremity edema. Urine studies revealed nephrotic range proteinuria and the immunological investigations were consistent with lupus. A renal biopsy revealed focal proliferative lupus nephritis. Conclusion. It is important to consider the presence of a coexisting autoimmune disease in a patient with sickle hemoglobinopathy who displays an atypical and multisystem presentation that is unresponsive to conventional therapies. When a significant kidney disease is present, a renal biopsy is critical in identifying the etiology of a renal abnormality in the setting of coexisting SLE and SCD. Vinay Minocha and Fauzia Rana Copyright © 2013 Vinay Minocha and Fauzia Rana. All rights reserved. Mayor Erythropoietic Response after Deferasirox Treatment in a Transfusion-Dependent Anemic Patient with Primary Myelofibrosis Wed, 06 Nov 2013 07:45:08 +0000 Primary myelofibrosis (PMF) is a myeloproliferative neoplasm frequently complicated by transfusion dependent anemia. Both anemia and transfusion-dependence are associated with a poor outcome, at least in part because of toxic effects of iron overload (IOL). Iron-chelating therapy (ICT) is increasingly used in order to prevent IOL in this setting. Here, we describe the case of a 73-year-old man affected by PMF and severe transfusion-dependent anemia who experienced a dramatic erythroid response after being treated with deferasirox to prevent IOL. Del Corso Lisette, Balleari Enrico, Arboscello Eleonora, Ghio Riccardo, Mencoboni Manlio, and Racchi Omar Copyright © 2013 Del Corso Lisette et al. All rights reserved. Low Molecular Weight Heparin Induced Skin Necrosis without Platelet Fall Revealing Immunoallergic Heparin Induced Thrombocytopenia Sun, 03 Nov 2013 00:00:00 +0000 Low molecular weight heparins (LMWH) are commonly used in the ICU setting for thromboprophylaxis as well as curative decoagulation as required during renal replacement therapy (RRT). A rare adverse event revealing immunoallergic LMWH induced thrombopenia (HIT) is skin necrosis at injection sites. We report the case of a patient presenting with skin necrosis witnessing an HIT after RRT, without thrombocytopenia. The mechanism remains unclear. Anti-PF4/heparin antibodies, functional tests (HIPA and/or SRA), and skin biopsy are of great help to evaluate differential diagnosis with a low pretest probability 4T’s score. Thomas Godet, Sébastien Perbet, Aurélien Lebreton, Guillaume Gayraud, Sophie Cayot, Aymeric Tremblay, Aurélie Ravinet, Sébastien Christophe, Renaud Guérin, Julien Pascal, Matthieu Jabaudon, Amr Hassan, Anne-Françoise Sapin, Jean-Etienne Bazin, and Jean-Michel Constantin Copyright © 2013 Thomas Godet et al. All rights reserved.