Case Reports in Hematology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2015 , Hindawi Publishing Corporation . All rights reserved. A Case of Fulminant Hepatitis due to Echovirus 9 in a Patient on Maintenance Rituximab Therapy for Follicular Lymphoma Thu, 28 May 2015 07:30:40 +0000 http://www.hindawi.com/journals/crihem/2015/454890/ Rituximab is a CD20 monoclonal antibody commonly used in the treatment of haematological malignancies. It causes lymphopenia with subsequent compromised humoral immunity resulting in an increased risk of infection. A number of infections and viral reactivations have been described as complicating Rituximab therapy. We report an apparently unique case of echovirus 9 (an enterovirus) infection causing an acute hepatitis and significant morbidity in an adult patient on maintenance treatment of Rituximab for follicular lymphoma. We also describe potential missed opportunities to employ more robust screening for viral infections which may have prevented delays in the appropriate treatment and thus may have altered the patient’s clinical course. We also make suggestions for lowering the threshold of viral testing in similar patients in the future. Ceri Morgan, S. J. Thomson, Joanne Legg, and Santosh Narat Copyright © 2015 Ceri Morgan et al. All rights reserved. Localized Relapse of Primary Plasma Cell Leukaemia in the Central Nervous System Sun, 10 May 2015 11:20:39 +0000 http://www.hindawi.com/journals/crihem/2015/273565/ Primary plasma cell leukaemia (pPCL) is a rare and aggressive form of plasma cell malignancies with a very poor prognosis. Compared to other plasma cell malignancies the tendency to extramedullary spread is increased; however central nervous system (CNS) involvement is rare and only reported in few cases. We report the case of a 61-year-old man who was diagnosed with pPCL and achieved a complete remission after autologous stem cell transplantation but had a relapse in the CNS without systemic disease. Christian W. Eskelund and Niels Frost Andersen Copyright © 2015 Christian W. Eskelund and Niels Frost Andersen. All rights reserved. AL Amyloidosis Complicated by Persistent Oral Bleeding Thu, 07 May 2015 06:56:13 +0000 http://www.hindawi.com/journals/crihem/2015/981346/ A case of amyloid light chain (AL) amyloidosis is presented here with uncontrolled bleeding after a nonsurgical dental procedure, most likely multifactorial in nature, and consequently treated with a multidisciplinary approach. Luiz Antonio Liarte Marconcini, Forrest Marc Stewart, Lisa Sonntag, Emily Stevens, and Nicholas Burwick Copyright © 2015 Luiz Antonio Liarte Marconcini et al. All rights reserved. Calreticulin Mutated Essential Thrombocythemia Presenting as Acute Coronary Syndrome Wed, 29 Apr 2015 08:31:21 +0000 http://www.hindawi.com/journals/crihem/2015/161764/ Essential thrombocythemia (ET) is a myeloproliferative neoplasm characterized by a clonal expansion of megakaryocytes. ET can result in both arterial and venous thrombosis. Involvement of the coronary arteries has been reported. Patients who harbor a CALR mutation are half as likely to suffer a thrombotic event as compared to patients with a JAK2 mutation. We report a case of CALR-mutated ET whose initial disease manifestation was a non-ST segment elevation myocardial infarction. Bassel Nazha, Gwenalyn Garcia, Ruben Kandov, and Marcel Odaimi Copyright © 2015 Bassel Nazha et al. All rights reserved. The Role of Inspiratory Muscle Training in Sickle Cell Anemia Related Pulmonary Damage due to Recurrent Acute Chest Syndrome Attacks Tue, 28 Apr 2015 12:47:05 +0000 http://www.hindawi.com/journals/crihem/2015/780159/ Background. The sickling of red blood cells causes a constellation of musculoskeletal, cardiovascular, and pulmonary manifestations. A 32-year-old gentleman with sickle cell anemia (SCA) had been suffering from recurrent acute chest syndrome (ACS). Aim. To examine the effects of inspiratory muscle training (IMT) on pulmonary functions, respiratory and peripheral muscle strength, functional exercise capacity, and quality of life in this patient with SCA. Methods. Functional exercise capacity was evaluated using six-minute walk test, respiratory muscle strength using mouth pressure device, hand grip strength using hand-held dynamometer, pain using Visual Analogue Scale, fatigue using Fatigue Severity Scale, dyspnea using Modified Medical Research Council Scale, and health related quality of life using European Organization for Research and Treatment of Cancer QOL measurement. Results. A significant improvement has been demonstrated in respiratory muscle strength, functional exercise capacity, pain, fatigue, dyspnea, and quality of life. There was no admission to emergency department due to acute chest syndrome in the following 12 months after commencing regular erythrocytapheresis. Conclusion. This is the first report demonstrating the beneficial effects of inspiratory muscle training on functional exercise capacity, respiratory muscle strength, pain, fatigue, dyspnea, and quality of life in a patient with recurrent ACS. Burcu Camcıoğlu, Meral Boşnak-Güçlü, Müşerrefe Nur Karadallı, Şahika Zeynep Akı, and Gülsan Türköz-Sucak Copyright © 2015 Burcu Camcıoğlu et al. All rights reserved. A Pelvic Pseudotumor in a Nonhemophilic Patient: An Unusual Presentation Mon, 27 Apr 2015 13:03:58 +0000 http://www.hindawi.com/journals/crihem/2015/359735/ Hemophilic pseudotumor is a rare complication of hemophilia, occurring in 1 to 2 percent of individuals with severe factor VIII or factor IX deficiency. A 35-year-old male presented with a swelling in the right lower abdomen for 3 months. There was no history of trauma. Examination revealed a swelling over the right iliac fossa. Right hip showed 30° flexion deformity. Blood investigations like complete blood count, APTT, PT, bleeding and clotting time, and fibrinogen were all normal. Plain radiograph and MRI showed a lytic lesion in the right iliac wing. Excision biopsy of the swelling showed organized hematoma with a fibrous capsule suggestive of a pseudotumor. Further haematological workup like factors VIII and IX was normal. At 2 years follow-up, there was no recurrence. We report this case of pseudotumour in patient without any bleeding disorder. Such case has not been reported in literature to the best of our knowledge. Mohamad Gouse, Abel Livingston, Dan Barnabas, and Vinoo Mathew Cherian Copyright © 2015 Mohamad Gouse et al. All rights reserved. Primary Lymphoma of Bone Presenting as Spindle Cell Neoplasm of the Vertebral Body: A Case Report and Review of the Literature Thu, 23 Apr 2015 11:22:11 +0000 http://www.hindawi.com/journals/crihem/2015/518307/ Spindle cell variant of lymphoma is a very rare but known disease entity that can mimic a sarcoma. Diagnosis can be even more challenging if the only site of the disease is in the bone. We report a case of primary lymphoma of bone with spindle cell morphology which was successfully treated with a combination of surgery, chemotherapy, and radiotherapy. Mahakit Inklab, Richard H. Steingart, and Jonathan K. Freeman Copyright © 2015 Mahakit Inklab et al. All rights reserved. Successful Aortic Aneurysm Repair in a Woman with Severe von Willebrand (Type 3) Disease Sun, 19 Apr 2015 17:01:28 +0000 http://www.hindawi.com/journals/crihem/2015/703803/ von Willebrand disease type 3 (VWD3) is a rare but the most severe form of von Willebrand disease; it is due to almost complete lack of von Willebrand factor activity (VWF:RCo). It is inherited as autosomal recessive trait; whilst heterozygote carriers have mild, or no symptoms, patients with VWD3 show severe bleeding symptoms. In the laboratory, this is characterised by undetectable VWF:Ag, VWF:RCo, and reduced levels of factor VIII < 0.02 IU/dL. The bleeding is managed with von Willebrand/FVIII factor concentrate replacement therapy. In this rare but challenging case we report on the successful excision and repair of an ascending aortic aneurysm following adequate VWF/FVIII factor concentrate replacement using Haemate-P. Victoria Campbell, Kevin Marriott, Rex Stanbridge, and Abdul Shlebak Copyright © 2015 Victoria Campbell et al. All rights reserved. Nonsecretory Multiple Myeloma Presenting as an Intestinal Tumor Thu, 16 Apr 2015 06:33:15 +0000 http://www.hindawi.com/journals/crihem/2015/818715/ We report a case of a 43-year-old Caucasian man who presented with colicky abdominal pain and microcytic hypochromic anemia. The patient underwent a colonoscopy where a tumor was seen in the ascending colon; histology showed plasmacytoma of the colon. From the protein electrophoresis, no monoclonal band or free light chains were detected nor was urinary Bence Jones protein present. A bone marrow biopsy showed plasma cell myeloma. To the best of our knowledge, this is the first case of nonsecretory multiple myeloma presenting as plasmacytoma of the colon. Diana Triantafyllopoulou, Stuart Mellor, Catherine Cargo, Ioannis Gkikas, Jagdish Adiyodi, Ayub Ali Bin, Neil Sahasrabudhe, and Margaret Rokicka Copyright © 2015 Diana Triantafyllopoulou et al. All rights reserved. Rare Form of Erdheim-Chester Disease Presenting with Isolated Central Skeletal Lesions Treated with a Combination of Alfa-Interferon and Zoledronic Acid Wed, 08 Apr 2015 12:46:51 +0000 http://www.hindawi.com/journals/crihem/2015/876752/ Erdheim-Chester disease (ECD) represents a clonal non-Langerhans histiocytosis, which manifests under an extensive variety of clinical symptoms. This creates a challenge for the physician, who is required to recognize and diagnose the disease in the early stages. Despite this considerable challenge, in the last decade there has been a dramatic increase in ECD diagnoses, in most part due to an increasing awareness of this rare disorder. Involvement of the axial skeleton is exclusively uncommon with no official recommendations for the treatment of the bone lesions. Here, we present a case report of a young male patient with isolated lesions of the spine, ribs, and pelvis, who was successfully treated with a combination therapy of alfa-interferon and zoledronic acid. E. N. Bulycheva, V. V. Baykov, M. I. Zaraĭskiĭ, and G. N. Salogub Copyright © 2015 E. N. Bulycheva et al. All rights reserved. Optimal Molecular Methods in Detecting p190BCR-ABL Fusion Variants in Hematologic Malignancies: A Case Report and Review of the Literature Wed, 08 Apr 2015 08:30:20 +0000 http://www.hindawi.com/journals/crihem/2015/458052/ Patients with BCR-ABL1 positive hematologic malignancies and Philadelphia-like B-lymphoblastic leukemia (B-ALL) are potential candidates for targeted therapy with tyrosine kinase inhibitors (TKI). Before TKIs, patients with B-ALL had a much worse prognosis and current treatments with targeted TKI therapy have improved outcomes. Thus, the detection of BCR-ABL1 is crucial and a false negative BCR-ABL1 result may adversely affect patient care. We report a case of a 76-year-old male with a new diagnosis of B-ALL who was initially found to be BCR-ABL1 negative by quantitative polymerase chain reaction (PCR). A concurrent qualitative PCR was performed which detected a positive BCR-ABL1 result that was confirmed by a next generation sequencing (NGS) based assay and identified as the rare fusion variant e1a3 of p. Based on this result, the patient was placed on dasatinib as a targeted therapy. In the era of molecular diagnostic medicine and targeted therapy, it is essential to have an understanding of the limitations of molecular assays and to follow a comprehensive diagnostic approach in order to detect common abnormalities and rare variants. Incorporating NGS methods in an algorithmic manner into the standard diagnostic PCR-based approach for BCR-ABL1 will aid in minimizing false negative results. Rebecca J. Sonu, Brian A. Jonas, Denis M. Dwyre, Jeffrey P. Gregg, and Hooman H. Rashidi Copyright © 2015 Rebecca J. Sonu et al. All rights reserved. Danazol: An Effective Option in Acquired Amegakaryocytic Thrombocytopaenic Purpura Sun, 05 Apr 2015 15:57:38 +0000 http://www.hindawi.com/journals/crihem/2015/171253/ Acquired amegakaryocytic thrombocytopaenic purpura (AATP) is a rare haematological condition characterised by isolated thrombocytopaenia with normal other cell lines. It is often initially misdiagnosed as immune thrombocytopaenic purpura but has characteristic bone marrow findings of reduced megakaryocyte numbers. The optimal treatment of AATP is not clearly defined but revolves around immunosuppressive therapies. We report a case of successful treatment of AATP with danazol, an antioestrogenic medication. We also review the aetiologies and pathogenesis of the disorder and suggest that danazol should be considered as an effective alternative to potent immunosuppression in AATP. E. Mulroy, S. Gleeson, and S. Chiruka Copyright © 2015 E. Mulroy et al. All rights reserved. Localized Lymph Node Light Chain Amyloidosis Thu, 02 Apr 2015 07:06:03 +0000 http://www.hindawi.com/journals/crihem/2015/816565/ Immunoglobulin-derived light chain amyloidosis can occasionally be associated with localized disease. We present a patient with localized lymph node light chain amyloidosis without an underlying monoclonal protein or lymphoproliferative disorder and review the literature of lymph node amyloidosis discussing work-up and risk factors for systemic progression. Binod Dhakal, Alexandra M. Harrington, Michael E. Stadler, and Anita D’Souza Copyright © 2015 Binod Dhakal et al. All rights reserved. Treatment of Acquired von Willebrand Syndrome and Prevention of Bleeding Postautologous Stem Cell Transplant during Severe Pancytopenia with IVIG Thu, 02 Apr 2015 06:56:43 +0000 http://www.hindawi.com/journals/crihem/2015/809313/ The use of high dose chemotherapy followed by autologous hematopoietic stem cell transplantation for remission consolidation after initial induction represents standard of care for patients with multiple myeloma. Patients with myeloma and Acquired von Willebrand Syndrome (AVWS) undergoing autologous stem cell transplant (ASCT) are at significant risk of bleeding due to the profound thrombocytopenia, low Factor VIII levels, fever, and toxicities associated with the preparative regimen. We report a patient with AVWS associated with multiple myeloma who underwent autologous stem cell transplants as consolidation after initial induction and again at relapse. He was successfully treated with high dose intravenous immunoglobulin (IVIG) prior to each transplant with rapid resolution of AVWS. Behyar Zoghi, Paul Shaughnessy, Roger M. Lyons, Richard Helmer III, Carlos Bachier, and C. Frederick LeMaistre Copyright © 2015 Behyar Zoghi et al. All rights reserved. Two Cases of Primary Cold Agglutinin Disease Associated with Megaloblastic Anemia Mon, 30 Mar 2015 10:44:13 +0000 http://www.hindawi.com/journals/crihem/2015/913795/ We report two cases of primary cold agglutinin disease (CAD) associated with megaloblastic anemia in Japanese elderly patients. Case 1 was a 67-year-old male and Case 2 was a 55-year-old male. Both patients were diagnosed with primary CAD, with continuously high cold agglutinin titers (1 : >8,192 and 1 : 16,834, resp.), monoclonal IgM-kappa light chains, and no underlying disease. In addition, both patients had megaloblastic anemia due to vitamin B12 deficiency. One patient received rituximab and both received vitamin 12 supplementation. To date, no cooccurrence of primary CAD and megaloblastic anemia has been emphasized. Thus, the association of these hematological diseases may be incidental; however, given that CAD is an autoimmune disease which may show antibodies against intrinsic factor and gastric parietal cells, this association was thought to be probably not a coincidence. Clinicians should be aware of the possible simultaneous presence of autoimmune hemolytic/megaloblastic anemia in patients with primary CAD. Shinsaku Imashuku, Naoko Kudo, Katsushige Takagishi, and Katsuyasu Saigo Copyright © 2015 Shinsaku Imashuku et al. All rights reserved. Durable Red Blood Cell Transfusion Independence in a Patient with an MDS/MPN Overlap Syndrome Following Discontinuation of Iron Chelation Therapy Mon, 30 Mar 2015 10:00:50 +0000 http://www.hindawi.com/journals/crihem/2015/253294/ Background. Hematologic improvement (HI) occurs in some patients with acquired anemias and transfusional iron overload receiving iron chelation therapy (ICT) but there is little information on transfusion status after stopping chelation. Case Report. A patient with low IPSS risk RARS-T evolved to myelofibrosis developed a regular red blood cell (RBC) transfusion requirement. There was no response to a six-month course of study medication or to erythropoietin for three months. At 27 months of transfusion dependence, she started deferasirox and within 6 weeks became RBC transfusion independent, with the hemoglobin normalizing by 10 weeks of chelation. After 12 months of chelation, deferasirox was stopped; she remains RBC transfusion independent with a normal hemoglobin 17 months later. We report the patient’s course in detail and review the literature on HI with chelation. Discussion. There are reports of transfusion independence with ICT, but that transfusion independence may be sustained long term after stopping chelation deserves emphasis. This observation suggests that reduction of iron overload may have a lasting favorable effect on bone marrow failure in at least some patients with acquired anemias. Harpreet Kochhar, Chantal S. Leger, and Heather A. Leitch Copyright © 2015 Harpreet Kochhar et al. All rights reserved. Lymph Node Flow Cytometry as a Prompt Recognition of Ultra Early Onset PTLD: A Successful Case of Rituximab Treatment Tue, 24 Mar 2015 13:49:50 +0000 http://www.hindawi.com/journals/crihem/2015/430623/ Ultra early posttransplantation lymphoproliferative disorder (PTLD) is a rare and fatal complication after hematopoietic stem cell transplantation (HSCT). Here we report, by lymph node (LN) flowcytometry, that we early recognized ultra early PTLD after an HLA-matched sibling allo-HSCT followed by a successful treatment with anti-CD20 antibody (rituximab) in a patient in progress disease for angioimmunoblastic T-cell lymphoma (AITL). The patient was conditioned with a reduced intensity conditioning (RIC) regimen. One week after transplantation, the patient developed high fever, generalized fatigue, high Epstein-Barr virus (EBV) load, and LN enlargement. An LN lymphocyte suspension and peripheral blood flowcytometry was performed to find majority of LN lymphocytes highly expressed CD20. By highly suspicious PTLD, 4 doses of rituximab (375 mg/m2 qw) were given immediately followed by reducing and withdrawing immunosuppressant reagent. PTLD was later confirmed by pathology. The patient had good response to rituximab, showing absence of fever, reduction in LN size, and no detectable EBV-DNA. Twenty months after HSCT, the patient remains well without evidence of AITL and PTLD. The current report is one of the earliest cases of PTLD after HSCT. Taken together, by LN flowcytometry as a prompt recognition, rituximab can be an effective preemptive therapy for ultra early developed PTLD. Xiaofan Li, Nainong Li, Ting Yang, Zhizhe Chen, and Jianda Hu Copyright © 2015 Xiaofan Li et al. All rights reserved. JAK2V617F: Is It Sufficient as a Single Player in Splanchnic Venous Thrombosis? Tue, 24 Mar 2015 11:36:41 +0000 http://www.hindawi.com/journals/crihem/2015/373490/ Splanchnic venous thrombosis (SVT) includes thrombosis of the hepatic, portal, and mesenteric venous system. Myeloproliferative neoplasms (MPNs) are important factors of SVT in adults. Addition of JAK2V617F mutation in WHO criteria for diagnosis of MPNs has made this test a useful tool for diagnosis. JAK2 is an intracytoplasmic tyrosine kinase that plays a critical role in signal transduction from multiple hematopoietic factor receptors. The mutation is found frequently in patients with SVT; many such patients have no other manifestations of an MPN. Although the correlation of JAK2V617F mutation with thrombotic risk in MPNs has been shown in many studies, the impact of presence of additional thrombophilic factors in these cases is yet not known. As the management of MPNs remains highly dependent on the patient’s thrombotic risk, it is important to assess the thrombotic risk factors in detail. Here, we report two cases of JAK2V617F positive MPN who also had other thrombophilic conditions and presented with recurrent thrombosis. Pratibha Dhiman and Priyanka Saxena Copyright © 2015 Pratibha Dhiman and Priyanka Saxena. All rights reserved. Nelarabine Associated Myotoxicity and Rhabdomyolysis Wed, 18 Mar 2015 10:39:27 +0000 http://www.hindawi.com/journals/crihem/2015/825670/ Nelarabine (ara-G; Arranon; compound 506U78) is an antineoplastic purine analog used for the treatment of refractory or relapsed T-cell acute lymphoblastic leukemia (T-ALL) and T-cell lymphoblastic lymphoma (T-LBL). The drug was granted accelerated approval in October 2005 by the US Food and Drug Administration (FDA) given the efficacy (induction of complete responses) noted in 2 single-arm trials (one in pediatric setting and one in adult patient population). The main spectra of toxicities that have been reported in these clinical trials and subsequent studies are hematological and neurological. Nelarabine induced rhabdomyolysis and increased creatinine phosphokinase (CK; CPK) levels apparently have been reported and this side effect has been added as an adverse reaction in the product monograph from the drug company during postmarketing surveillance. However, the true extent and incidence of the myotoxicity from the drug are unclear. In this paper we report a grade IV CK elevation and rhabdomyolysis in a patient with T-ALL treated with nelarabine. Given the reported finding, we examined the literature further for myotoxicity, increased CK, and/or rhabdomyolysis associated with the use of the nelarabine and report our findings. Mahnur Haider, Syed Ahsan Rizvi, and Pashtoon Murtaza Kasi Copyright © 2015 Mahnur Haider et al. All rights reserved. Virus-Associated Hemophagocytic Syndrome in Renal Transplant Recipients: Report of 2 Cases from a Single Center Sun, 08 Mar 2015 14:15:10 +0000 http://www.hindawi.com/journals/crihem/2015/876301/ Virus-associated hemophagocytic syndrome (HPS) is a potentially fatal complication of immunosuppression for transplantation. However, it presents with heterogeneous clinical symptoms (fever, disturbed consciousness, and hepatosplenomegaly) and laboratory findings (pancytopenia, elevated hepatic enzyme levels, abnormal coagulation, and hyperferritinemia), impeding diagnosis. Case 1: A 39-year-old female developed fever 4 years after ABO-incompatible living-related renal transplantation. Laboratory findings revealed thrombocytopenia, elevated hepatic enzymes, Epstein-Barr virus (EBV) DNA seropositivity, and hyperferritinemia. EBV-associated HPS was confirmed by bone marrow aspiration. Steroid pulse therapy and etoposide were ineffective. Disseminated intravascular coagulation resulted in multiple organ failure, and the patient died 32 days after disease onset. Case 2: A 67-year-old male was admitted with rotavirus enteritis a month after living-unrelated renal transplantation. He developed sudden-onset high fever, disturbance of consciousness, and tachypnea 8 days after admission. Laboratory findings revealed elevated hepatic enzyme levels, hyperkalemia, and hyperferritinemia. Emergency continuous hemodiafiltration ameliorated the fever, and steroid pulse therapy improved abnormal laboratory values. Varicella-zoster virus meningitis was confirmed by spinal tap. Acyclovir improved consciousness, and he was discharged 87 days after admission. Fatal virus-associated HPS may develop in organ transplant patients receiving immunosuppressive therapy. Pathognomonic hyperferritinemia is useful for differential diagnosis. Koji Nanmoku, Takayuki Yamamoto, Makoto Tsujita, Takahisa Hiramitsu, Norihiko Goto, Akio Katayama, Shunji Narumi, Yoshihiko Watarai, Takaaki Kobayashi, and Kazuharu Uchida Copyright © 2015 Koji Nanmoku et al. All rights reserved. A Complicated Case of Acute Promyelocytic Leukemia in the Second Trimester of Pregnancy Successfully Treated with All-trans-Retinoic Acid Mon, 02 Mar 2015 12:01:32 +0000 http://www.hindawi.com/journals/crihem/2015/634252/ A 40-year-old female at 26-week gestation was diagnosed with acute promyelocytic leukemia (APL) after an abnormal prenatal lab workup showed pancytopenia. She was treated with all-trans-retinoic acid (ATRA), idarubicin, and dexamethasone. After day one of treatment, she developed differentiation syndrome, which was treated with dexamethasone. At 30-week gestation, she had preterm premature rupture of membranes and delivered by cesarean section because of the fetus’ breech presentation. Despite ATRA’s potential for teratogenicity, a viable infant was born without apparent anomalies. Postpartum, she underwent consolidation treatment with ATRA and arsenic trioxide (ATO). The patient continued ATRA therapy after delivery and is currently in remission. Kanika Agarwal, Megha Patel, and Vandana Agarwal Copyright © 2015 Kanika Agarwal et al. All rights reserved. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation Wed, 18 Feb 2015 12:35:33 +0000 http://www.hindawi.com/journals/crihem/2015/201536/ Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH) presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8) vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented. B. Routy, J. Hoang, and J. Gruber Copyright © 2015 B. Routy et al. All rights reserved. Transformation of an Unclassified Myeloproliferative Neoplasm with a Rare BCR-JAK2 Fusion Transcript Resulting from the Translocation (9;22)(p24;q11) Wed, 18 Feb 2015 06:35:18 +0000 http://www.hindawi.com/journals/crihem/2015/252537/ BCR-ABL1 negative myeloproliferative neoplasms (MPNs) are known to contain alterations of the tyrosine kinase JAK2 (located on 9p24) that result in constitutive activation of the encoded protein. JAK2 fusions are reported in acute and chronic leukemias of myeloid and lymphoid phenotypes. Here, we report an unclassified case of MPN (MPN-U) showing a t(9;22)(p24;q11), which generates a BCR-JAK2 fusion gene by fusing the BCR at intron 13 to JAK2 at intron 17 on the derivative chromosome 22. Most reported JAK2 fusions cases reveal an aggressive clinical course and long-term remissions have only been achieved after allogeneic stem cell transplantation (ASCT). To the best of our knowledge, this is the thirteenth case reported worldwide to describe a BCR-JAK2 fusion transcript in MPN-U. The present report revealed a sustained complete clinical, hematologic, and cytogenetic remission 35 months after diagnosis and ~24 months after ASCT. Regarding BCR-ABL1  negative MPN patients this case report provides strong support for a role of JAK2 activation in the oncogenesis and suggests a possible diagnostic and therapeutic target that should be investigated. A. N. Chamseddine, P. Etancelin, D. Penther, F. Parmentier, C. Kuadjovi, V. Camus, N. Contentin, P. Lenain, C. Bastard, H. Tilly, and F. Jardin Copyright © 2015 A. N. Chamseddine et al. All rights reserved. Hyperhemolysis Syndrome without Underlying Hematologic Disease Tue, 17 Feb 2015 09:42:51 +0000 http://www.hindawi.com/journals/crihem/2015/180526/ Introduction. Hyperhemolysis is characterized by a life-threatening hemolytic transfusion reaction, with hemoglobin (Hb) and hematocrit (Hct) dropping markedly lower than before transfusion. This phenomenon, commonly described in sickle cell disease, is a rare occurrence in patients without hemoglobinopathies. Case Report. A 55-year-old male presented to the hospital after a motorcycle crash and received 10 units of cross-matched blood for active bleeding. The patient was blood group O, with a negative antibody screen. Ten days later, he represented complaining of dyspnea and was found to have a hematocrit of 12%. The direct antiglobulin test was positive for anti-immunoglobin G and complement. Indirect antiglobulin test was positive for anti-Jka alloantibodies. The presence of Jka antigen was revealed in one unit of previously transfused blood; patient’s RBCs were negative for the Jka antigen. Laboratory data demonstrated findings consistent with DHTR, as well as reticulopenia and elevated ferritin levels. He continued to show signs of active hemolysis, requiring a total of 4 subsequent units of pRBCs. Each transfusion precipitated a drop in Hb and Hct to levels lower than before transfusion; once transfusions were held, the patient slowly recovered. Discussion. Hyperhemolysis in the setting of a DHTR can occur in patients without hematologic disease. Lauren Anne Eberly, Diaa Osman, and Nathaniel Perryman Collins Copyright © 2015 Lauren Anne Eberly et al. All rights reserved. A Proven Case of Cutaneous Rhizopus Infection Presenting with Severe Limb Pain Very Soon after Induction Treatment in a Patient with Acute Lymphoblastic Leukemia Sun, 15 Feb 2015 12:55:46 +0000 http://www.hindawi.com/journals/crihem/2015/285360/ Objective and Importance. Invasive mucormycosis may complicate the course of patients with hematologic malignancies and has a very high mortality rate. Early diagnosis and aggressive approach combined with surgical and medical treatment have paramount importance for cure. Clinical Presentation. We report here a case of a patient with acute lymphoblastic leukemia presenting with a subcutaneous mass lesion which was sampled by an ultrasound guided needle biopsy. The pathology showed microorganisms with aseptate hyphae with wide, irregular walls and more or less branching with highly vertical angles which suggested a mold infection. The specimen was also cultured where Rhizopus spp. grew. Conclusion. Posaconazole 200 mg QID was commenced. She recovered from neutropenia and pain on day 20 of treatment. After 4 courses of hyper-CVAD chemotherapy, the remaining soft tissue mass was removed surgically and she underwent allogeneic HSCT from a full matched sibling donor under secondary prophylaxis. Mehmet Sezgin Pepeler, Kadir Acar, Özlem Güzel Tunçcan, Ömer Uluoğlu, Ayşe Kalkancı, Hakan Atalar, Koray Kılıç, and Gülsan Türköz Sucak Copyright © 2015 Mehmet Sezgin Pepeler et al. All rights reserved. Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care Setting Tue, 10 Feb 2015 10:46:31 +0000 http://www.hindawi.com/journals/crihem/2015/491567/ Hemophagocytic lymphohistiocytosis (HLH) is a rare and potential life-threatening clinical syndrome that results from uncontrolled activation of the immune system. Secondary HLH, more commonly observed in adult patients, is seen in the context of underlying triggering conditions. Epstein-Barr virus (EBV) has been recognized as the leading infectious cause and is associated with a poor outcome. As clinical and laboratory features of HLH could overlap with septic shock syndrome in most patients, the diagnosis of HLH, especially in adults, is the most challenging aspect of the disease that results in delayed recognition and treatment of rapidly progressive multiorgan system failure. We report a case of Hemophagocytic lymphohistiocytosis in a patient who presented with signs of septic shock syndrome and we review the literature on the topic. Neda Hashemi-Sadraei, Pimprapa Vejpongsa, Muhamed Baljevic, Lei Chen, and Modupe Idowu Copyright © 2015 Neda Hashemi-Sadraei et al. All rights reserved. Serum Total Tryptase Level Confirms Itself as a More Reliable Marker of Mast Cells Burden in Mast Cell Leukaemia (Aleukaemic Variant) Tue, 10 Feb 2015 08:15:33 +0000 http://www.hindawi.com/journals/crihem/2015/737302/ Mast cell leukemia (MCL) is a very rare form of systemic mastocytosis (SM) with a short median survival of 6 months. We describe a case of a 65-year-old woman with aleukaemic variant of MCL with a very high serum total tryptase level of 2255 μg/L at diagnosis, which occurred following an episode of hypotensive shock. She fulfilled the diagnostic criteria of SM, with a bone marrow smear infiltration of 50–60% of atypical mast cells (MCs). She tested negative for the KIT D816V mutation, without any sign of organ damage (no B- or C-findings) and only few mediator-related symptoms. She was treated with antihistamine alone and then with imatinib for the appearance of anemia. She maintained stable tryptase level and a very indolent clinical course for twenty-two months; then, she suddenly progressed to acute MCL with a serum tryptase level up to 12960 μg/L. The patient died due to haemorrhagic diathesis twenty-four months after diagnosis. This clinical case maybe represents an example of the chronic form of mast cell leukemia, described as unpredictable disease, in which the serum total tryptase level has confirmed itself as a reliable marker of mast cells burden regardless of the presence of other signs or symptoms. P. Savini, M. Rondoni, G. Poletti, A. Lanzi, O. Quercia, S. Soverini, C. De Benedittis, G. Musardo, G. Martinelli, and G. F. Stefanini Copyright © 2015 P. Savini et al. All rights reserved. Renal and Neurological Response with Eculizumab in a Patient with Transplant Associated Thrombotic Microangiopathy after Allogeneic Hematopoietic Progenitor Cell Transplantation Thu, 29 Jan 2015 12:12:04 +0000 http://www.hindawi.com/journals/crihem/2015/425410/ Transplantation-associated thrombotic microangiopathy (TA-TMA) is a challenge after allogeneic hematopoietic progenitor cell transplantation, considering the diagnostic uncertainties and lack of established treatment. We report a 43-year-old male patient who was diagnosed as TA-TMA after allogeneic progenitor cell transplantation for a progressive ALK negative anaplastic large cell lymphoma and responded to eculizumab with dramatically improving neurological status and renal function. Rapid neurological and renal recovery achieved after eculizumab could support a possible relationship between complement activation and TA-TMA. Eculizumab should be a reasonable treatment approach in patients with TA-TMA after allogeneic hematopoietic progenitor cell transplantation. Ömür Gökmen Sevindik, İnci Alacacıoğlu, Abdullah Katgı, Şerife Medeni Solmaz, Celal Acar, Özden Pişkin, Mehmet Ali Özcan, Fatih Demirkan, Bülent Ündar, and Güner Hayri Özsan Copyright © 2015 Ömür Gökmen Sevindik et al. All rights reserved. Long Complete Remission Achieved with the Combination Therapy of Cisplatin and Gemcitabine in a Patient with Aggressive Natural Killer Cell Leukemia Wed, 28 Jan 2015 09:31:59 +0000 http://www.hindawi.com/journals/crihem/2015/715615/ Aggressive natural killer cell leukemia (ANKL) is a rare and often lethal lymphoproliferative disorder. Patients may present with constitutional symptoms, jaundice, skin infiltration, lymphadenopathy, and hepatosplenomegaly. ANKL can progress quickly to multiorgan failure and survival is usually measured in weeks. Although a rapid and accurate diagnosis is critical, unfortunately there is no hallmark diagnostic marker of ANKL. We report a case of a 48-year-old male who was able to obtain a complete remission following cisplatin-based chemotherapy. We describe the details of the chemotherapy regimens used and a literature review of the treatment of ANKL. Justin LaPorte, Lawrence Morris, and John Koepke Copyright © 2015 Justin LaPorte et al. All rights reserved. Relapse of Multiple Myeloma Presenting as Extramedullary Plasmacytomas in Multiple Organs Wed, 28 Jan 2015 07:44:11 +0000 http://www.hindawi.com/journals/crihem/2015/452305/ Multiple myeloma is a neoplastic plasma cell disorder. It is characterized by collections of abnormal plasma cells accumulating in the bone marrow, where they interfere with the production of normal blood cells. It usually presents as a multisystemic involvement, whose symptoms and signs vary greatly. Some patients have slowly progressive disease while others have aggressive clinical behavior by extramedullary involvement. In addition to renal failure, anemia, hypercalcemia, lytic bone lesions, and immunodeficiency, it also affects multiple organ system, such as pancreas, adrenal glands, kidney, skin, lung, liver, spleen, lymph nodes, and bone. To raise awareness of the variable presentations of this disease, we report a 53-year-old male patient, with multiple myeloma in his first remission who relapsed with extramedullary plasmacytomas (EMPs) involving multiple organs, such as pancreas, adrenal glands, kidney, skin, lung, liver, spleen, and lymph nodes. Murat Köse, Ersida Buraniqi, Timur Selçuk Akpinar, Seyit Mehmet Kayacan, and Tufan Tükek Copyright © 2015 Murat Köse et al. All rights reserved.