Case Reports in Hematology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2013 , Hindawi Publishing Corporation . All rights reserved. Mycobacterium avium Complex-Associated Hemophagocytic Lymphohistiocytosis in a Sickle Cell Patient: An Unusual Fatal Association Thu, 16 May 2013 10:27:14 +0000 http://www.hindawi.com/crim/hematology/2013/291518/ Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome, characterized clinically by fever, splenomegaly, cytopenia, and high ferritin. Infectious causes have been associated with secondary HLH, with viruses being the most common. We report a case of Mycobacterium avium complex-associated HLH in a sickle cell anemia patient. To the best of our knowledge, this association has never been reported in sickle cell anemia. Mohammed A. R. Chamsi-Pasha, M. Chadi Alraies, Abdul Hamid Alraiyes, and Eric D. Hsi Copyright © 2013 Mohammed A. R. Chamsi-Pasha et al. All rights reserved. Vascular Endothelial Cell Function in Catastrophic Antiphospholipid Syndrome: A Case Report and Review of the Literature Wed, 15 May 2013 17:31:12 +0000 http://www.hindawi.com/crim/hematology/2013/710365/ Catastrophic antiphospholipid syndrome (CAPS) is a rare autoimmune condition, which has been associated with a high mortality rate. However, with current management that includes a combination of anticoagulation, glucocorticoid administration, and plasma exchange, mortality rate has declined. Despite survival improvement with new generation immunosuppressive agents, their mechanisms of action are poorly defined, and CAPS is still considered a high-risk complication in patients known with antiphospholipid antibody syndrome. Herein, we present a case of a 79-year-old male who presented with a myocardial infarct and renal failure secondary to CAPS following a splenectomy for immune thrombocytopenia. Regardless of rapid combination of first-line treatment and rituximab therapy, the patient developed lethal cardiogenic shock secondary to mitral valve papillary muscle necrosis. Discussion of the pathophysiology and avenues of future therapies in CAPS are reported. B. Routy, T. Huynh, R. Fraser, and C. Séguin Copyright © 2013 B. Routy et al. All rights reserved. An Uncommon Cause of Gastrointestinal Bleeding in an 84-Year-Old Female Sun, 12 May 2013 18:52:35 +0000 http://www.hindawi.com/crim/hematology/2013/940271/ The estimated annual incidence for drug-induced thrombocytopenia is 10 per million. Although fatal consequences are uncommon, life-threatening hemorrhage can occur due to spontaneous bleeding. We report a case of 84-year-old Caucasian female who presented to the emergency department with multiple episodes of bloody bowel movements. One week prior to this admission, she was started on trimethoprim-sulfamethoxazole for the treatment of skin abscess. On admission laboratory results showed platelet count of 4 × 103/mm3 and hemoglobin of 10.2 g/dL. Because of unstable vital signs, the patient was transferred to the intensive care unit where she received multiple units of platelet and blood transfusion. Drug-induced thrombocytopenia due to TMP/SMX was suspected. Intravenous methylprednisolone was started as well as immune globulin with good clinical response. Suartcha Prueksaritanond, Aram Barbaryan, Alaa M. Ali, and Aibek E. Mirrakhimov Copyright © 2013 Suartcha Prueksaritanond et al. All rights reserved. Mitomycin-C-Induced TTP/HUS Treated Successfully with Rituximab: Case Report and Review of the Literature Sun, 12 May 2013 17:58:35 +0000 http://www.hindawi.com/crim/hematology/2013/130978/ Microangiopathic hemolytic anemia (MAHA), thrombocytopenia, fever, renal failure, and neurologic symptoms comprise the cardinal features of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Etiologies can include medications, infections, cancers, or transplantation. We present a patient with a history of rectal cancer treated with mitomycin-C who developed MAHA, acute kidney injury, and thrombocytopenia 6 months after completing therapy and to did not respond the plasmapheresis or steroids. She was treated with four weekly doses of rituximab with full recovery. Gunjan Shah, Hanah Yamin, and Hedy Smith Copyright © 2013 Gunjan Shah et al. All rights reserved. Erratum to “A Case of Abdominal Sarcoidosis in a Patient with Acute Myeloid Leukemia” Sun, 12 May 2013 17:08:55 +0000 http://www.hindawi.com/crim/hematology/2013/240545/ Vadsala Baskaran, Amanda Goodwin, Lavanya Athithan, Alfredo Addeo, and Ciro Rinaldi Copyright © 2013 Vadsala Baskaran et al. All rights reserved. A Unique “Composite” PTLD with Diffuse Large B-Cell and T/Anaplastic Large Cell Lymphoma Components Occurring 17 Years after Transplant Tue, 07 May 2013 18:56:12 +0000 http://www.hindawi.com/crim/hematology/2013/386147/ Posttransplant lymphoproliferative disorder (PTLD) comprises a spectrum ranging from polyclonal hyperplasia to aggressive monoclonal lymphomas. The majority of PTLDs are of B-cell origin while T-cell PTLDs and Hodgkin lymphoma-like PTLDs are uncommon. Here, we report a unique case of a 56-year-old man in whom a lymphoma with two distinct components developed as a duodenal mass seventeen years following a combined kidney-pancreas transplant. This PTLD, which has features not previously reported in the literature, consisted of one component of CD20 positive and EBV negative monomorphic diffuse large B-cell lymphoma. The other component showed anaplastic morphology, expressed some but not all T-cell markers, failed to express most B-cell markers except for PAX5, and was diffusely EBV positive. Possible etiologies for this peculiar constellation of findings are discussed and the literature reviewed for “composite-like” lymphomas late in the posttransplant setting. Kristin La Fortune, Dahua Zhang, Gordana Raca, and Erik A. Ranheim Copyright © 2013 Kristin La Fortune et al. All rights reserved. Incidental Discovery of a Testicular Plasmacytoma at Initial Presentation of Multiple Myeloma Tue, 07 May 2013 11:39:51 +0000 http://www.hindawi.com/crim/hematology/2013/752921/ Testicular plasmacytomas are a rare phenomenon reported in the literature and they can occur as a solitary plasmacytoma, as a recurrence of multiple myeloma, or concurrently in an active myeloma. We report the case of a 43-year-old man who presented with back pain and was diagnosed with multiple myeloma. A CT scan performed to determine the extent of disease revealed an incidental mass in the testicle. Immunohistochemical staining of the mass revealed monoclonal cytolpasmic IgA in the tumor cells and serum studies showed this same immunoglobulin. Following orchiectomy, radiotherapy to the vertebra, chemotherapy with bortezomib, dexamethasone, and doxorubicin, and an autologous bone marrow transplant, the patient is alive twelve months after diagnosis and is in complete remission. Amanda R. Hathaway Copyright © 2013 Amanda R. Hathaway. All rights reserved. Thrombotic Thrombocytopenic Purpura: A Case Presenting with Acute Ischemic Colitis Tue, 07 May 2013 08:32:45 +0000 http://www.hindawi.com/crim/hematology/2013/592930/ Thrombotic thrombocytopenic purpura (TTP) consists of the pentad of thrombocytopenia, hemolytic anemia, fever, neurologic abnormalities, and renal disease. We present a case report of acute TTP following a bout of ischemic colitis. This report reminds the clinician that ischemic colitis can be an atypical presentation of TTP. The prompt recognition and treatment of this disease process resulted in a good prognosis for our patient. Joseph R. H. See, Tarek Sabagh, and Christopher J. Barde Copyright © 2013 Joseph R. H. See et al. All rights reserved. Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation Therapy Thu, 02 May 2013 13:28:06 +0000 http://www.hindawi.com/crim/hematology/2013/428297/ Background. Rosai-Dorfman Disease (RDD) is rare benign hematologic disorder of histiocytes, which usually manifests as painless lymphadenopathy, fever, leukocytosis and hypergammaglobulinemia. Extranodal RDD has been reported in 43% of cases, with skin as commonly involved site followed by head and neck region. However, soft tissue cheek mass is rare presentation of extra-nodal RDD. Case Presentation. A 26-year-old Saudi man presented with a six-month history of right cheek swelling and left upper eyelid swelling. Physical examination revealed right cheek mass of size  cm and left upper eyelid mass of size  cm and no palpable cervical lymphadenopathy. Incisional biopsy of cheek mass showed positivity for S100 and negativity for CD1a, consistent with extra-nodal RDD. Patient did not respond to systemic steroids and was given radiation therapy to left orbit with minimal response. Then, he was started on chemotherapy Rituximab, cyclophosphamide, vincristine, and prednisolone (RCVP) 8 cycles followed by reirradiation 10 Gy in 10 fractions with 6 MeV electron with complete response. After 12 months of followup, patient had recurrence in right cheek and was started on radiotherapy to the cheek mass. Conclusion. Extra-nodal RDD with involvement of cheek is a rare presentation. Incorporation of S100 and CD1a is helpful in diagnoses of RDD and differentiating it from other benign histiocytosis. Treatment consists of surgery, steroids, chemotherapy, and radiation therapy. Ahmed Marzouk Maklad, Yasser Bayoumi, Mutahir Tunio, Wafaa AlShakweer, Mashooque A. Dahar, and Shomaila A. Akbar Copyright © 2013 Ahmed Marzouk Maklad et al. All rights reserved. Autoimmune Hemolytic Anemia and Nodular Lymphocyte-Predominant Hodgkin Lymphoma: A Rare Association Sun, 28 Apr 2013 17:44:28 +0000 http://www.hindawi.com/crim/hematology/2013/567289/ Autoimmune hemolytic anemia (AIHA) has been associated with chronic lymphocytic leukemia, non-Hodgkin lymphoma, and classical Hodgkin lymphoma, but to the best of our knowledge, the association of AIHA and nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) has not been reported previously. A 20-year-old woman presented with conjunctival jaundice, fever, asthenia, and hemoglobin 9.2 g/dL revealing IgG-mediated warm antibody AIHA. Computed tomography (CT) scan and positron-emission tomography (PET) scan showed mediastinal and axillary lymph nodes with increased [18F]-fluorodeoxyglucose uptake. A mediastinal lymph node was biopsied during mediastinoscopy, and NLPHL was diagnosed by an expert hematopathologist. The hemoglobin level declined to 4.6 g/dL. The treatment consisted of four 28-day cycles of R-ABVD (rituximab 375 mg/m2 IV, adriamycin 25 mg/m2 IV, bleomycin 10 mg/m2 IV, vinblastine 6 mg/m2 IV, and dacarbazine 375 mg/m2 IV, each on days 1 and 15). Prednisone was progressively tapered over 10 weeks. After the first chemotherapy cycle, the hemoglobin level rose to 12 g/dL. After the four cycles, PET and CT scans showed complete remission (CR). At the last followup (4 years), AIHA and NLPHL were in sustained CR. Géraldine Salmeron, Thierry Jo Molina, Claire Fieschi, Anne-Marie Zagdanski, Pauline Brice, and David Sibon Copyright © 2013 Géraldine Salmeron et al. All rights reserved. Ibuprofen-Induced Hemolytic Anemia Sun, 21 Apr 2013 15:41:55 +0000 http://www.hindawi.com/crim/hematology/2013/142865/ Drug-induced immune hemolytic anemia is a rare condition with an incidence of 1 per million of the population. We report the case of a 36-year-old female who presented to the emergency department complaining of shortness of breath and dark colored urine. Physical examination was significant for pale mucous membranes. The patient reported using ibuprofen for a few days prior to presentation. Complete blood count performed before starting ibuprofen revealed normal platelets and hemoglobin values. On admission, the patient had evidence of hemolytic anemia with hemoglobin of 4.9 g/dL, hematocrit of 14.2%, lactate dehydrogenase 435 IU/L, and reticulocytosis 23.2%. Further testing ruled out autoimmune disease, lymphoma, and leukemia as etiologies for the patient’s new onset hemolytic anemia. Ibuprofen was immediately stopped with a gradual hematologic recovery within 3 days. Aram Barbaryan, Chioma Iyinagoro, Nwabundo Nwankwo, Alaa M. Ali, Raya Saba, Shawn G. Kwatra, Nasir Hussain, Chukwuemeka C. Uzoka, Suartcha Prueksaritanond, and Aibek E. Mirrakhimov Copyright © 2013 Aram Barbaryan et al. All rights reserved. Clonal Hypereosinophilia with ETV6 Rearrangement Evolving to T-Cell Lymphoblastic Lymphoma: A Case Report and Review of the Literature Sun, 21 Apr 2013 15:37:43 +0000 http://www.hindawi.com/crim/hematology/2013/652745/ Hypereosinophilia, either clonal or reactive, has been described in association with multiple hematological malignancies. We describe a case of a patient presenting with hypereosinophilia that evolved into T-cell lymphoblastic lymphoma. Complete remission was achieved with chemotherapy; however, hypereosinophilia recurred 5 months later in association with myeloblastic bone marrow infiltration and without evidence of lymphoblastic lymphoma relapse. Cytogenetic analysis of the bone marrow showed a complex translocation involving chromosomes 7, 12, and 16. A rearrangement of ETV6 gene (12p13) was demonstrated by FISH studies, thus confirming the clonality of this population. The association of lymphoblastic lymphoma, eosinophilia, and myeloid hyperplasia has been described in disorders with FGFR1 rearrangements. We hypothesize that other clonal eosinophilic disorders lacking this rearrangement could behave in a similar fashion through different pathogenic mechanisms. Filipa Moita, Isabel Bogalho, Helena Alaiz, Joana Parreira, Maria Jesus Frade, Albertina Nunes, and Maria Gomes da Silva Copyright © 2013 Filipa Moita et al. All rights reserved. Small-Sized Clone of T Cells in Multiple Myeloma Patient after Auto-SCT: T-LGL Leukemia Type or Clonal T-Cell Aberration? Sun, 21 Apr 2013 11:26:36 +0000 http://www.hindawi.com/crim/hematology/2013/417353/ Second cancers and particularly postransplant lymphoproliferative disorders (PTLDs) are extremely rare in patients undergoing autologous peripheral blood stem cell transplantation (auto-SCT). We report the case of clonally rearranged T-cell expansion which occurred after auto-SCT for Multiple Myeloma (MM). Does asymptomatic clonal T-cell large granular lymphocytic proliferation, in our experience, represent either a secondary cancer after auto-SCT or clonal T cell aberration or derive from expansion of coexisting undetected small-sized clone of T cells? Giuseppe Mele, Marilena Greco, Maria Rosaria Coppi, Giacomo Loseto, Angela Melpignano, Salvatore Mauro, and Gianni Quarta Copyright © 2013 Giuseppe Mele et al. All rights reserved. Detection of Rare Beta Globin Gene Mutation [+22 5UTR(G>A)] in an Infant, Despite Prenatal Screening Sun, 14 Apr 2013 17:12:14 +0000 http://www.hindawi.com/crim/hematology/2013/906292/ Background. Beta thalassemia is one of the most common hereditary disorders worldwide. In Iran, it is frequently reported from northern and southern provinces. In order to prevent child birth to be affected by this complication, prenatal screening and diagnosis are carried out nationwide. However, in some instances, this program is unable to identify rare mutations leading to thalassemia. Case Presentation. A married couple, who took part in prenatal screening and diagnosis, gave birth to a child who is affected by thalassemia major. After several molecular examinations, a rare mutation [+22 5UTR (G>A)] in compound heterozygote state along with a common mutation [codon 8 (-AA)] was found. Conclusion. This case study suggests that more advanced molecular evaluations must be integrated in prenatal screening programs to identify rare mutations and antenatal diagnosis of thalassemia cases. Mohammad Reza Mahdavi, Hosein Karami, Mohammad Taghi Akbari, Hosein Jalali, and Payam Roshan Copyright © 2013 Mohammad Reza Mahdavi et al. All rights reserved. BRAF V600E-Negative Hairy Cell Leukaemia Tue, 09 Apr 2013 13:34:39 +0000 http://www.hindawi.com/crim/hematology/2013/513049/ Since the initial report of the BRAF V600E mutation in hairy cell leukemia, numerous investigators have demonstrated the presence of this activating mutation in nearly all cases of this disease. A case of hairy cell leukemia is documented with a classical clinical, morphological, immunophenotypic, and cytochemical profile in which the BRAF V600E was not detected. The diagnostic and therapeutic implications are discussed. Stephen E. Langabeer, David O'Brien, Anthony M. McElligott, Michelle Lavin, and Paul V. Browne Copyright © 2013 Stephen E. Langabeer et al. All rights reserved. Refractory Anemia with Ring Sideroblasts Associated with Marked Thrombocytosis Complicated by Massive Splenomegaly Treated with Lenalidomide Resulting in Resolution of Splenomegaly but Severe and Prolonged Pancytopenia Sun, 07 Apr 2013 15:05:14 +0000 http://www.hindawi.com/crim/hematology/2013/718480/ Refractory anemia with ring sideroblasts associated with marked thrombocytosis (RARS-T) is a hematological malignancy that combines features of both a myeloproliferative and myelodysplastic disorder. There have been recent reports of the successful treatment of anemia in 2 patients with RARS-T with lenalidomide. Here we report the successful treatment of massive splenomegaly in a patient with a long history of RARS-T resulting in complete resolution of splenomegaly, but with prolonged severe cytopenias. We also report the acquisition of the t(3;12)(q26;p13) translocation previously described in cases of myelodysplasia and the potential for transformation to myelofibrosis. Gordon Taylor, Dominic Culligan, and Mark A. Vickers Copyright © 2013 Gordon Taylor et al. All rights reserved. Allogeneic Hematopoietic Cell Transplantation for Therapy-Related Myeloid Leukemia following Orthotopic Cardiac Transplantation Sat, 30 Mar 2013 12:11:05 +0000 http://www.hindawi.com/crim/hematology/2013/140138/ Therapy-related myeloid neoplasm (t-MN) is a subtype of acute myeloid leukemia with adverse cytogenetics and poor overall prognosis despite intensive induction chemotherapy and allogeneic hematopoietic cell transplantation (allo-HCT). It is increasingly recognized as a late complication of chronic immunosuppression in patients who have received solid organ transplantation. In this paper, we describe a case of t-MN following orthotopic cardiac transplantation and its treatment with allo-HCT. We discuss molecular and biological challenges and considerations in double solid organ and bone marrow transplantation and review similar cases at our institution. Our experience suggests general feasibility and safety of allo-HCT in patients who have received solid organ transplantation. Richard J. Lin, Richard A. Larson, Koen van Besien, and Elizabeth S. Rich Copyright © 2013 Richard J. Lin et al. All rights reserved. Successful Use of Cyclophosphamide as an Add-On Therapy for Multiple Myeloma Patients with Acquired Resistance to Bortezomib or Lenalidomide Thu, 28 Mar 2013 09:14:04 +0000 http://www.hindawi.com/crim/hematology/2013/651902/ Novel agents such as thalidomide, lenalidomide, and bortezomib have been shown to possess potent activity against multiple myeloma. However, the treatment strategy for patients who acquired resistance to these agents has not been established. In addition to switching drug classes, intensified treatment strategy, including increase in the dosage of current agents and addition of other agents, may be considered for these patients. We here describe 2 myeloma patients with acquired resistance to bortezomib or lenalidomide, in whom add-on therapy with low-dose cyclophosphamide was effective and tolerable. These cases suggest that add-on therapy with cyclophosphamide is one of the treatment options to overcome resistance to novel agents in patients with multiple myeloma. A larger prospective study is needed to clarify the efficacy and safety of this strategy for novel agent-resistant multiple myeloma. Shigeki Ito, Tatsuo Oyake, Kazunori Murai, and Yoji Ishida Copyright © 2013 Shigeki Ito et al. All rights reserved. High-Output Cardiac Failure Revealing Primary Plasma Cell Leukemia Mon, 04 Mar 2013 13:58:11 +0000 http://www.hindawi.com/crim/hematology/2013/504612/ High-output cardiac failure in multiple myeloma (MM) is related to arteriovenous shunting in bone infiltrate disease. We describe such a complication in a patient with primary plasma cell leukemia (pPCL) without bone disease. We review the mechanisms that could be involved. As previously described, traditional cardiac failure therapy is not effective. pPCL therapy should not be delayed. Driss Chaoui, Bruno Gallet, Philippe Genet, Babette Mbungani, Ahmad Al Jijakli, Nina Arakelyan, Louisa Mesbah, and Laurent Sutton Copyright © 2013 Driss Chaoui et al. All rights reserved. Postpartum Acquired Hemophilia: A Rare Cause of Postpartum Hemorrhage Thu, 28 Feb 2013 14:50:45 +0000 http://www.hindawi.com/crim/hematology/2013/735715/ A 36-year-old female started having postpartum vaginal bleeding after normal vaginal delivery. She underwent hysterectomy for persistent bleeding and was referred to our institution. An elevation of PTT and normal PT made us suspect postpartum acquired hemophilia (PAH), and it was confirmed by low factor VIII activity levels and an elevated factor VIII inhibitor. Hemostasis was achieved with recombinant factor VII concentrates and desmopressin, and factor eradication was achieved with cytoxan, methylprednisolone, and plasmapheresis. Srikanth Seethala, Sumit Gaur, Elizabeth Enderton, and Javier Corral Copyright © 2013 Srikanth Seethala et al. All rights reserved. A Case of Abdominal Sarcoidosis in a Patient with Acute Myeloid Leukemia Thu, 28 Feb 2013 09:00:20 +0000 http://www.hindawi.com/crim/hematology/2013/379898/ The allogeneic bone marrow transplantation usually preceded by induction chemotherapy, in fit patients, represents the gold standard in the acute myeloid leukaemia. In the last years, many trials have been set up with the view of improving the number of remissions during the induction by adding new drugs. Several early or late side effects have been described in the literature. We herein present a patient with acute myeloid leukaemia patient who, after chemotherapy, developed ascites that turned out to be abdominal sarcoidosis. Vadsala Baskaran, Amanda Goodwin, Lavanya Athithan, Ciro Roberto Rinaldi, and Alfredo Addeo Copyright © 2013 Vadsala Baskaran et al. All rights reserved. Bendamustine Associated with Irreversible Ascending Paralysis Wed, 27 Feb 2013 14:31:03 +0000 http://www.hindawi.com/crim/hematology/2013/931519/ Bendamustine is an alkylating agent currently used in the treatment of lymphoproliferative disorders. Many adverse effects, including a rare case of reversible neurotoxicity, have been reported in association with bendamustine. Herein, we report the first case of irreversible ascending paralysis related to bendamustine. Ashraf Alhafez, Omar S. Aljitawi, Tara L. Lin, Siddhartha Ganguly, Sunil Abhyankar, and Joseph P. McGuirk Copyright © 2013 Ashraf Alhafez et al. All rights reserved. Clinical Manifestations and Diagnostic Challenges in Acute Porphyrias Tue, 12 Feb 2013 08:54:54 +0000 http://www.hindawi.com/crim/hematology/2013/628602/ The porphyrias are a group of disorders characterized by an enzyme deficiency in the heme biosynthetic pathway. These can be classified into either erythropoietic or hepatic forms depending on the site of the major enzyme deficiency. The diagnosis of acute porphyrias, however, can be very challenging due to overlapping features amongst the various types. Initial suspicion is based on a myriad of clinical manifestations, which then are confirmed by laboratory testing where available. Genetic testing is now also available for the different types of porphyrias, aiding in the definitive diagnosis. Here, we present a challenging case of porphyria in a patient with end-stage renal disease and present the diagnostic challenges associated with the case and the ways forward. Henry Trier, Vikram P. Krishnasamy, and Pashtoon Murtaza Kasi Copyright © 2013 Henry Trier et al. All rights reserved. Cutaneous Plasmablastic Lymphoma in an Immunocompetent Patient with Long-Term Pyrimethamine Use for Essential Thrombocythemia: A Case Report and Literature Review Wed, 06 Feb 2013 16:02:33 +0000 http://www.hindawi.com/crim/hematology/2013/541783/ We report a case of Epstein-Barr-virus-(EBV-) positive primary cutaneous plasmablastic lymphoma in a human-immunodeficiency-virus-(HIV-) negative, immunocompetent 62-year-old female patient. We postulate that her lymphoma development is due to the longstanding use of pyrimethamine for essential thrombocythemia. This has never been described in the literature. Ing Soo Tiong, Magreet Strauss, Michael B. Y. Lau, and Shingirai Chiruka Copyright © 2013 Ing Soo Tiong et al. All rights reserved. Perioperative Management of Type 2N Von Willebrand’s Disease with Recombinant Factor VIII in a Patient Undergoing Knee-Replacement Surgery Sun, 03 Feb 2013 14:23:20 +0000 http://www.hindawi.com/crim/hematology/2013/837906/ Type 2N Von Willebrand's disease (2N VWD) is a rare, recessively inherited bleeding disorder, comprising 1-2% of all VWD patients, usually manifesting as a mild bleeding diathesis. Treatment includes desmopressin (DDAVP) or intermediate purity plasma-derived FVIII concentrates containing residual VWF. We present a case of a 75-year-old gentleman, incidentally diagnosed with type 2N VWD in 2002 on routine blood testing before surgery with an ISTH bleeding score of 1-2, who was treated with recombinant FVIII preoperatively. Srivasavi Dukka and David John Allsup Copyright © 2013 Srivasavi Dukka and David John Allsup. All rights reserved. t(17;19) in Children with Acute Lymphocytic Leukemia: A Report of 3 Cases and a Review of the Literature Thu, 03 Jan 2013 15:27:50 +0000 http://www.hindawi.com/crim/hematology/2013/563291/ Several cytogenetic abnormalities identified in patients with childhood acute lymphocytic leukemia (ALL) have been associated with a poor prognosis. There are several case reports in the literature describing t(17;19) in children with ALL. This translocation has been associated with hypercalcemia, coagulopathy, and poor outcome. We present three cases of ALL with t(17;19) treated at our institution and review the outcome of children reported in the medical literature. Katherine A. Minson, Pinki Prasad, Susan Vear, Scott Borinstein, Richard Ho, Jennifer Domm, and Haydar Frangoul Copyright © 2013 Katherine A. Minson et al. All rights reserved. Deep Vein Thrombosis, Raynaud's Phenomenon, and Prinzmetal Angina in a Patient with Glanzmann Thrombasthenia Mon, 31 Dec 2012 18:18:25 +0000 http://www.hindawi.com/crim/hematology/2012/156290/ Patients with Glanzmann thrombasthenia fail to form large platelet thrombi due to mutations that affect the biosynthesis and/or function of the αIIbβ3 integrin. The result is a moderate to severe bleeding syndrome. We now report unusual vascular behaviour in a 55-year-old woman with classic type I disease (with no platelet αIIbβ3 expression) and a homozygous ITGA2B missense mutation (E324K) affecting the terminal β-propeller domain of αIIb. While exhibiting classic bleeding symptoms as a child, in later life this woman first developed deep vein thrombosis after a long air flight then showed vascular problems characteristic of Raynaud’s phenomenon, and finally this year she presented with chest pains suggestive of coronary heart disease. Yet while coronary angiography first showed a stenosis, this was not seen on a second examination when she was diagnosed with coronary spastic angina and Prinzmetal phenomenon. It is significant that the absence of platelet aggregation with physiologic agonists had not prevented any of the above cardiovascular or vascular diseases. Alan Nurden, Patrick Mercié, Pascal Zely, and Paquita Nurden Copyright © 2012 Alan Nurden et al. All rights reserved. Treated Follicular Lymphoma, Recurrent Invasive Pneumococcal Disease, Nonresponsiveness to Vaccination, and a Unique Pneumococcus Sun, 30 Dec 2012 12:05:12 +0000 http://www.hindawi.com/crim/hematology/2012/386372/ A nonneutropenic patient with treated low-grade non-Hodgkin’s (Follicular) lymphoma and secondary hypogammaglobulinemia recovered from pneumococcal pneumonia and septicemia (serotype 7F; ST191) subsequent to influenza A H1N1 (2009). Both infections were potentially vaccine preventable. The patient then developed pneumococcal meningitis due to a serotype 35F pneumococcus with a unique Multilocus Sequence Type (ST7004) which was not vaccine preventable. Patient management was influenced by host predisposition to pneumococcal infection, antibiotic intolerance, and poor response to polysaccharide pneumococcal vaccine. Indirect immunofluorescence with anti-human immunoglobulin confirmed a poor or intermediate response to Pneumovax II. Prophylactic erythromycin was initiated, and immunoglobulin transfusions were also commenced as a preventive strategy. ST7004 is a single locus variant of ST1635 which has been associated with the serotype 35F capsule in England. The spi gene in ST7004, which differentiates it from ST1635, is the same as the spi gene present in ST191 which could have arisen from the first disease episode suggesting that horizontal gene transfer may have occurred between different populations of pneumococci present within the patient in an attempt to evade vaccination selection pressure. Clare Murphy, Donald Inverarity, Claire McGoldrick, Lindsay Mitchell, Pamela Paterson, Louise Thom, and Giles Edwards Copyright © 2012 Clare Murphy et al. All rights reserved. A Platelet Acquired Storage Pool Disorder Associated with Tamoxifen Therapy Wed, 26 Dec 2012 18:49:57 +0000 http://www.hindawi.com/crim/hematology/2012/948351/ The antiestrogenic drug tamoxifen, used in patients with breast cancer, is associated with an increase in arterial and venous thrombotic events, the mechanism of which is not clearly understood. We report a case of a lady who presented with new bruising and prolonged bleeding following a tooth extraction 4–6 weeks after starting tamoxifen. Investigations were consistent with an acquired platelet storage pool disorder. Repeat platelet function analysis was normal, performed 3 months after discontinuation of tamoxifen. We present a previously clinically unreported effect of tamoxifen on platelet function. Lalitha Nayak and Alvin H. Schmaier Copyright © 2012 Lalitha Nayak and Alvin H. Schmaier. All rights reserved. Fatal Hepatocellular Carcinoma in a Patient with Occult Hepatitis B Virus Infection following the Administration of R-CHOP for Diffuse Large B-Cell Lymphoma Wed, 26 Dec 2012 15:07:11 +0000 http://www.hindawi.com/crim/hematology/2012/803298/ Occult hepatitis B (OBI) is caused by a persistent low-level replication of HBV. Like overt HBV infection, OBI can be associated with the integration of HBV sequences into the host genome and has a substantial clinical relevance for patients who are severely immunosuppressed for long durations. We present the case of a patient with a diffuse large B-cell non-Hodgkin lymphoma and OBI who developed a hepatocellular carcinoma with a fulminant clinical course following the administration of rituximab plus CHOP. Vincenzo Pitini, Massimo Rizzo, Carmela Arrigo, Patrizia Mondello, and Giuseppe Altavilla Copyright © 2012 Vincenzo Pitini et al. All rights reserved.