Case Reports in Hematology https://www.hindawi.com The latest articles from Hindawi © 2017 , Hindawi Limited . All rights reserved. Dasatinib and Prednisolone Induction Therapy for a Case of Philadelphia Chromosome-Positive Acute Lymphoblastic Leukemia with Dilated Cardiomyopathy Accompanied by Life-Threatening Ventricular Tachycardia Thu, 23 Feb 2017 08:22:45 +0000 http://www.hindawi.com/journals/crihem/2017/4027908/ A 56-year-old man being treated for dilated cardiomyopathy presented with epigastralgia. He was diagnosed with ventricular tachycardia and Philadelphia chromosome-positive acute lymphoblastic leukemia. After treating incessant ventricular tachycardia, we commenced induction therapy for leukemia with dasatinib and prednisolone to minimize toxicity towards cardiomyocytes and the cardiac conduction system. Although dasatinib was temporarily withheld because of a recurrence of ventricular tachycardia, we rechallenged dasatinib while using bisoprolol and amiodarone and achieved a complete hematological response three weeks later. Although drug interactions between dasatinib and amiodarone were of concern, the blood concentration of each drug remained within the safe range after concomitant use, and there were no adverse cardiac effects such as QT prolongation after rechallenging dasatinib. Induction therapy with dasatinib and prednisolone may be an acceptable therapeutic option for Philadelphia chromosome-positive acute lymphoblastic leukemia with severe cardiac complications. Mitsutaka Nishimoto, Hirohisa Nakamae, Kana Matsumoto, Kunihiko Morita, Yuki Koga, Dai Momose, and Masayuki Hino Copyright © 2017 Mitsutaka Nishimoto et al. All rights reserved. A Child as a Donor for Hematopoietic Stem Cell Transplantation: Bioethical Justification—A Case Study on Sickle Cell Disease Thu, 23 Feb 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/8394732/ Hematopoietic stem cell transplantation (HSCT) is an important treatment option for children with severe and refractory sickle cell disease (SCD) with debilitating clinical complications. HSCT with cells from the bone marrow of a HLA-identical sibling used in SCD has a low mortality risk, high cure rate, and high event-free survival rate after a median follow-up of 5-6 years. However, matched donors are found in only about 20% of the patients. A boy aged 8 years with SCD had a sister, <2 years old, a fully compatible donor. The boy met all eligibility criteria to undergo HSCT, and he was suffering from cognitive and neurologic impairment due to ischemic events. A Bioethical Committee jointly discussed the ethical issues on this case after a pediatric evaluation released the very young sister for donation. The justification was that the sister would benefit from the donation too because of the greater likelihood of survival and cure and less suffering of her brother. The parents were informed about the risks and benefits for both children, and the family was psychologically evaluated. After their consent, HSCT was performed and the patient is cured from SCD. The complication for the donor was the need for blood transfusion. Andrea Z. Pereira, Ricardo Hellman, Nelson Hamerschlak, Andrea Kondo, Polianna Mara Rodrigues de Souza, Wilson Leite Pedreira Jr., Luiz Fernando Alves Lima Mantovani, Eduardo Juan Troster, Henrique Grunspun, and Marco Aurélio Scarpinella Bueno Copyright © 2017 Andrea Z. Pereira et al. All rights reserved. Uncovering Clinical Features of De Novo Philadelphia Positive Myelodysplasia Tue, 21 Feb 2017 08:45:35 +0000 http://www.hindawi.com/journals/crihem/2017/5404131/ Myelodysplastic syndrome (MDS) is cytogenetically heterogeneous and retains variable risk for acute myeloid leukemia transformation. Though not yet fully understood, there is an association between genetic abnormalities and defects in gene expression. The functional role for infrequent cytogenetic alteration remains unclear. An uncommon chromosomic abnormality is the presence of the Philadelphia (Ph) chromosome. Here, we report a patient with Ph+ MDS treated with low dose Dasatinib who achieved hematologic response for 7 months. In addition, we also examined the English literature on all de novo Ph + MDS cases between 1996 and 2015 to gain insight into clinical features and outcome. Aristides Armas, Chen Chen, Martha Mims, and Gustavo Rivero Copyright © 2017 Aristides Armas et al. All rights reserved. Spontaneous Remission of an Untreated, MYC and BCL2 Coexpressing, High-Grade B-Cell Lymphoma: A Case Report and Literature Review Tue, 21 Feb 2017 07:41:24 +0000 http://www.hindawi.com/journals/crihem/2017/2676254/ Non-Hodgkin lymphomas (NHL) are a heterogeneous group of hematologic malignancies typically treated with multiagent chemotherapy. Rarely, spontaneous remissions can be observed, particularly in more indolent subtypes. The prognosis of aggressive NHL can be predicted using clinical and histopathologic factors. In aggressive B-cell NHL, the importance of MYC and BCL2 proto-oncogene coexpression (as assessed by immunohistochemistry) and high-grade histologic features are particularly noteworthy. We report a unique case of spontaneous remission in a patient with an aggressive B-cell NHL which harbored high-risk histopathologic features, including MYC protein expression at 70–80%, BCL2 protein expression, and morphologic features suggestive of high-grade B-cell lymphoma, NOS (formerly B-cell lymphoma unclassifiable with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma [BCLU]). After undergoing a biopsy to confirm this diagnosis, he opted to forego curative-intent chemotherapy. The single, yet relatively large area of involvement noted on 18F-fluorodeoxyglucose positron emission tomography-computed tomography steadily resolved on subsequent follow-up studies. He remained without evidence of recurrence one year later, having never received treatment. This case emphasizes the potential for spontaneous remission in NHL and demonstrates that this phenomenon can be observed despite contemporary high-risk histopathologic features. D. Alan Potts, Jonathan R. Fromm, Ajay K. Gopal, and Ryan D. Cassaday Copyright © 2017 D. Alan Potts et al. All rights reserved. Malignant Lymphoma in the Psoas Major Muscle Mon, 20 Feb 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3902748/ An 84-year-old Japanese man taking warfarin to prevent cerebral infarction secondary to atrial fibrillation was admitted to our hospital for evaluation of a painless right back mass. Magnetic resonance imaging (MRI) showed an oval-shaped mass in the right psoas major muscle. The mass showed high intensity on T1-, T2-, and diffusion-weighted imaging and mimicked an acute-phase hematoma. However, it showed no chronological changes typical of a hematoma, and MRI revealed enlargement of the mass 1 week after admission. Histopathological examination of a biopsy specimen revealed diffuse large B-cell lymphoma (DLBCL). Although skeletal muscle lymphoma is rare, physicians should be familiar with its MRI characteristics. In addition, determination of the lymphoma subtype has important implications for the treatment of skeletal muscle lymphoma because DLCBL may have an especially poor prognosis. Nobuhiro Akuzawa, Takashi Hatori, Aya Takase, Jun Aoki, Shinji Sakurai, and Masahiko Kurabayashi Copyright © 2017 Nobuhiro Akuzawa et al. All rights reserved. Donor-Derived Smoldering Multiple Myeloma following a Hematopoietic Cell Transplantation for AML Mon, 20 Feb 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3728429/ Posttransplant Lymphoproliferative Disorder (PTLD) is one of the most common malignancies complicating solid organ transplantation. In contrast, PTLD accounts for a minority of secondary cancers following allogeneic hematopoietic cell transplantation (HCT). Here we report on a 61-year-old woman who received an ABO-mismatched, HLA-matched unrelated donor hematopoietic cell transplantation from a presumably healthy donor for a diagnosis of acute myeloid leukemia (AML). Eighteen months following her transplant, she developed a monoclonal gammopathy. Bone marrow studies revealed 10% plasma cells, but the patient lacked clinical defining features of multiple myeloma (MM); thus a diagnosis of smoldering multiple myeloma (SMM) was established. Cytogenetic and molecular studies of the bone marrow confirmed the plasma cells were donor-derived. The donor lacks a diagnosis of monoclonal gammopathy of undetermined significance, SMM, or MM. Bita Fakhri, Mark Fiala, Michael Slade, Peter Westervelt, and Armin Ghobadi Copyright © 2017 Bita Fakhri et al. All rights reserved. Utility of MYD88 in the Differential Diagnosis and Choice of Second-Line Therapy in a Case of Nonsecretory Lymphoplasmacytic Lymphoma versus Free Light Chain Waldenstrom’s Macroglobulinemia Sun, 12 Feb 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/5183646/ The MYD88 L265P somatic variant (MYD88) has a high prevalence in Waldenstrom’s Macroglobulinemia (WM), a form of lymphoplasmacytic lymphoma (LPL) associated with monoclonal IgM. Although the role of MYD88 in WM was initially reported in 2012, it was not until 2016 that MYD88 testing was included in the National Cancer Care Network (NCCN) Guidelines. We present a case illustrating the utility of MYD88 status in distinguishing atypical forms of WM from marginal zone lymphoma (MZL) and in selecting second-line therapy with ibrutinib. In 2012, a 64-year-old male presented with dyspnea on exertion, a hemoglobin of 5.6 g/dL, a platelet count of 86,000, and monoclonal IgM kappa on serum immunofixation but no detectable M-spike. Bone marrow biopsy revealed 95% monoclonal B-lymphocytes with lymphoplasmacytic differentiation favoring a diagnosis of LPL/WM over MZL, with a favorable response to chemotherapy. This diagnosis was called into question 3 years later following relapse, and MZL was favored based on the lack of MYD88 mutation. One year later, however, repeat bone marrow biopsy detected the MYD88 mutation and therapy with ibrutinib yielded a favorable response. The distinction between certain lymphomas can be problematic and in this case MYD88 was helpful in clarifying a diagnosis of atypical LPL/WM from MZL and in selecting effective second-line therapy. D. Kazmierski, M. L. Palomba, and C. Barsigian Copyright © 2017 D. Kazmierski et al. All rights reserved. HHV8/EBV Coinfection Lymphoproliferative Disorder: Rare Entity with a Favorable Outcome Thu, 09 Feb 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/1578429/ HHV8/EBV-associated germinotropic lymphoproliferative disorder (GLD) is a challenging diagnosis given its rarity, the particular clinical presentation, and the lack of expression of markers usually used in establishing hematopoietic lineage. We report a new case of HHV8/EBV GLD in an immunocompetent 78-year-old woman. The diagnosis was made in an incidentally discovered lymphadenopathy. Histological examination showed a nodular lymphoid proliferation centered by aggregates of atypical plasmablastic cells admixed with small lymphoid cells. Tumor cells were strongly positive with EMA, HHV8, LMP1, CD38, CD138, and kappa light chains. They were negative with common lymphoma-associated markers (CD20, CD3, CD15, CD30, CD10, and bcl2). In situ hybridization confirmed the monotypic kappa light chains and the EBV infection (EBER+). A polyclonal pattern of Ig gene rearrangement was detected by PCR analysis. In the adjacent lymph node parenchyma, some germinal centers mimicked Castleman disease. In this case, the differential diagnosis was discussed with an early stage of large B-cell lymphoma arising in HHV8-associated multicentric Castleman disease. The clinical presentation, the immunophenotype, and the molecular results helped to make the accurate diagnosis. Through the review of the nine previously reported cases in literature, we discuss the clinical and pathologic features and the differential diagnosis of HHV8/EBV GLD. Dhouha Bacha, Beya Chelly, Houda Kilani, Lamia Charfi, Amel Douggaz, Samia Chatti, and Emna Chelbi Copyright © 2017 Dhouha Bacha et al. All rights reserved. Synchronous Occurrence of Chronic Myeloid Leukemia and Mantle Cell Lymphoma Wed, 08 Feb 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/7815095/ Chronic myeloid leukemia (CML) and mantle cell lymphoma (MCL) are hematologic malignancies that originate from different oligopotent progenitor stem cells, namely, common myeloid and lymphoid progenitor cells, respectively. Although blastic transformation of CML can occur in the lymphoid lineage and CML has been related to non-Hodgkin lymphoma on transformation, to our knowledge, de novo and synchronous occurrence of CML and MCL has not been reported. Herein, we report the first case of synchronous CML and MCL in an otherwise healthy 38-year-old man. Potential etiologies and pathological relationships between the two malignancies are explored, including the possibility that the downstream effects of BCR-ABL may link it to an overexpression of cyclin D1, which is inherent to the etiology of MCL. Prajwol Pathak, Ying Li, Brian Allen Gray, William Stratford May Jr., and Merry Jennifer Markham Copyright © 2017 Prajwol Pathak et al. All rights reserved. Spontaneous Heparin-Induced Thrombocytopenia and Venous Thromboembolism following Total Knee Arthroplasty Sun, 05 Feb 2017 07:40:12 +0000 http://www.hindawi.com/journals/crihem/2017/4918623/ A 72-year-old Caucasian woman was admitted for an elective left total knee arthroplasty. Her surgery was uncomplicated and she was discharged to a rehabilitation facility. Twelve days later, she developed acute shortness of breath followed by a syncopal episode. She was hypoxic and cyanotic, requiring hospitalization and intubation, and was subsequently diagnosed with bilateral submassive pulmonary emboli and bilateral lower extremity deep vein thrombosis. She was started on unfractionated heparin infusion. Within 24 hours of exposure, she had an acute decrease in platelet count to 48,000. Heparin was discontinued and argatroban was initiated due to concern for heparin-induced thrombocytopenia (HIT). Both quantitative enzyme immunoassay and functional assay confirmed the diagnosis of HIT. The patient had no prior lifetime heparin exposure. Given the absence of preceding heparin therapy, this case is consistent with the diagnosis of spontaneous HIT. Kevin Baker and Ming Y. Lim Copyright © 2017 Kevin Baker and Ming Y. Lim. All rights reserved. Myelodysplastic Syndrome Clinically Presenting with the “Classic TTP Pentad” Wed, 01 Feb 2017 11:10:21 +0000 http://www.hindawi.com/journals/crihem/2017/4619406/ The clinical presentation of myelodysplastic syndrome (MDS) is not specific. Many patients can be asymptomatic and can be detected only due to an abnormal complete blood cell count (CBC) on routine exam or for other reasons while others can be symptomatic as a consequence of underlying cytopenias. Thrombotic thrombocytopenic purpura (TTP) usually is suspected under the evidence of microangiopathic hemolytic anemia (MAHA) and thrombocytopenia and because it is a life-threatening condition (medical emergency) immediate initiation of plasmapheresis could be life-saving. The following case illustrates an unusual presentation of MDS in a patient who came in to the emergency room with the classic TTP “pentad” of fever, renal involvement, MAHA, mental status changes, and thrombocytopenia. We will focus our discussion in the clinical presentation of this case. Santiago Fabián Moscoso Martínez, Evelyn Carolina Polanco Jácome, Elizabeth Guevara, and Vijay Mattoo Copyright © 2017 Santiago Fabián Moscoso Martínez et al. All rights reserved. Allogeneic Transplant in ELANE and MEFV Mutation Positive Severe Cyclic Neutropenia: Review of Prognostic Factors for Secondary Severe Events Wed, 18 Jan 2017 09:58:59 +0000 http://www.hindawi.com/journals/crihem/2017/5375793/ Objective and Importance. Cyclic neutropenia (CyN) is a rare autosomal dominant inherited disorder due to the mutation ELANE primarily affecting bone marrow stem cells and is characterized by recurrent neutropenia every 2 to 4 weeks. Symptoms vary from benign to severe, including death. Postulations on the cause of wide spectrum in symptom presentation include the possibility of other genetic mutations, such as MEFV. Recommended treatment for CyN is G-CSF to keep ANC higher to minimize risk of infection. Case. A 25-year-old male diagnosed with CyN, on G-CSF but worsening quality of life. Pretransplant investigations revealed ELANE mutation positive severe CyN along with familial Mediterranean fever (MEFV) mutation. Intervention. Bone marrow transplantation as treatment for dual mutation (ELANE and MEFV mutation) positive severe CyN. Conclusion. BMT may be considered as an alternative treatment for severe CyN in patients who are refractory to G-CSF. It is postulated that in our patient the combined mutations (CyN and MEFV) may have contributed to the severity of this individual’s symptoms. We suggest CyN patients who present with severe symptoms have evaluation with ELANE mutation testing, Periodic Fever Syndromes Panel, and routine marrow assessment with FISH, conventional cytogenetics, and morphological evaluation for MDS/AML. Onyemaechi N. Okolo, Emmanuel Katsanis, Seongseok Yun, Candace Y. Reveles, and Faiz Anwer Copyright © 2017 Onyemaechi N. Okolo et al. All rights reserved. Hodgkin Lymphoma and Castleman Disease: When One Blood Disease Can Hide Another Wed, 18 Jan 2017 09:39:24 +0000 http://www.hindawi.com/journals/crihem/2017/9423205/ We describe a rare case of Castleman disease associated de novo with Hodgkin lymphoma. The incidence of Castleman disease is rare; only a few studies have described it in de novo association with Hodgkin lymphoma. The patient described here complained of unique evolutionary axillary adenopathy. A positron-emission tomography/computed tomography scan revealed hypermetabolic activity in this area. Diagnosis was based on a total excision biopsy of the adenopathy. The patient underwent complete remission with ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) chemotherapy for treating Hodgkin lymphoma after surgical excision of the unicentric Castleman disease lesion. L. Filliatre-Clement, H. Busby-Venner, C. Moulin, G. Roth-Guepin, and A. Perrot Copyright © 2017 L. Filliatre-Clement et al. All rights reserved. Angioimmunoblastic T-Cell Lymphoma: A Questionable Association with Follicular Dendritic Cell Sarcoma Wed, 18 Jan 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/9601094/ An elderly woman presented with generalized lymphadenopathy, several systemic symptoms, and splenomegaly. An inguinal lymph node excision revealed a compound picture. One aspect of the lymph node morphology, including cells with follicular T-helper cell phenotype, was most consistent with angioimmunoblastic T-cell lymphoma. The other component, revealing spindle cells forming whorls with immunostaining for CD21, CD23, and fascin, might be an integral part of this T-cell lymphoma. However, due to the often massive involvement of the nodal tissue by these follicular dendritic cells, these areas were questionably suggestive of involvement by follicular dendritic cell sarcoma. We raise herein the issue of the borderline area between advanced follicular dendritic cell expansion in angioimmunoblastic T-cell lymphoma and a massive follicular dendritic cell proliferation consistent with follicular dendritic cells sarcoma, in the absence of a genomic analysis. Daniel Benharroch, Miriam Zekzer, and Karen Nalbandyan Copyright © 2017 Daniel Benharroch et al. All rights reserved. Management of Venous Thromboembolism in Patients with Hereditary Antithrombin Deficiency and Pregnancy: Case Report and Review of the Literature Tue, 10 Jan 2017 12:26:35 +0000 http://www.hindawi.com/journals/crihem/2017/9261351/ Background. Hereditary antithrombin deficiency is a thrombogenic disorder associated with a 50–90% lifetime risk of venous thromboembolism (VTE), which is increased during pregnancy and the puerperium in these patients. We present a case of a woman with antithrombin (AT) deficiency who presented with a VTE despite therapeutic low molecular weight heparin (LMWH). Though the pregnancy was deemed unviable, further maternal complications were mitigated through the combined use of therapeutic anticoagulation and plasma-derived antithrombin concentrate infusions to normalize her functional antithrombin levels. Methods. A review of the literature was conducted for studies on prophylaxis and management of VTE in pregnant patients with hereditary AT deficiency. The search involved a number of electronic databases, using combinations of keywords as described in the text. Only English language studies between 1946 and 2015 were included. Conclusion. Antithrombin concentrate is indicated in pregnant women with hereditary AT deficiency who develop VTE despite being on therapeutic dose anticoagulation. Expert opinion suggests AT concentrate should be used concomitantly with therapeutic dose anticoagulation. However, further high-quality studies on the dose and duration of treatment in the postpartum period are required. Use of AT concentrate for prophylaxis is controversial and should be based on individual VTE risk stratification. Mohammad Refaei, Lydia Xing, Wendy Lim, Mark Crowther, and Kochawan Boonyawat Copyright © 2017 Mohammad Refaei et al. All rights reserved. Rapid Change in Mental Status in a Patient with Hypereosinophilia Tue, 10 Jan 2017 08:58:19 +0000 http://www.hindawi.com/journals/crihem/2017/6936709/ We present the case of a 48-year-old female with acute onset altered mental status, who was found to have eosinophilia, elevated troponin, and embolic strokes. Extensive testing for autoimmune, infectious, and coronary artery etiologies was unremarkable. After a cardiac MRI revealed focal myocardial hyperenhancement, the patient underwent an endomyocardial biopsy with findings consistent with eosinophilic myocarditis. The patient was diagnosed of idiopathic hypereosinophilic syndrome and started on prednisone and apixaban. Our case highlights the importance of considering hypereosinophilic syndrome when eosinophilia is associated with multisystem impairments, as tissue biopsy is usually required to diagnose this rare condition. Hanyin Wang and John K. Erban Copyright © 2017 Hanyin Wang and John K. Erban. All rights reserved. Radiation for MALT of the Submandibular Gland Mon, 09 Jan 2017 09:07:00 +0000 http://www.hindawi.com/journals/crihem/2017/8397621/ We are reporting a case of a 27-year-old woman with a history of swelling in the left submandibular region. This swelling was associated with a mass, and this was pathologically confirmed to be an extranodal marginal zone lymphoma (MALT). The patient underwent surgical excision and postoperative adjuvant radiation therapy. The patient tolerated treatments well and remains free of disease. Here, we describe the case and management described in the current literature. Juskaran Chadha, Marita S. Teng, Julie Teruya-feldstein, and Richard L. Bakst Copyright © 2017 Juskaran Chadha et al. All rights reserved. Gelatinous Marrow Transformation Associated with Imatinib: Case Report and Literature Review Wed, 04 Jan 2017 09:37:48 +0000 http://www.hindawi.com/journals/crihem/2017/1950724/ Gelatinous marrow transformation (GMT) is a rare condition observed in severe illness or malnutrition, in which the bone marrow contains amorphous “gelatinous” extracellular material, and histopathology demonstrates varied degrees of fat cell atrophy and loss of hematopoietic elements. An association of GMT with imatinib use in chronic myeloid leukemia (CML) has been reported recently. The objective of this study is to describe a case of GMT associated with imatinib use and review the existing similar cases in the literature to identify epidemiological patterns and potential imatinib-induced mechanisms leading to gelatinous conversion. E. Chang, G. Rivero, B. Jiang, S. Yellapragada, and P. Thiagarajan Copyright © 2017 E. Chang et al. All rights reserved. Secondary Hemochromatosis due to Chronic Oral Iron Supplementation Wed, 04 Jan 2017 06:22:50 +0000 http://www.hindawi.com/journals/crihem/2017/2494167/ Iron may accumulate in excess due to a mutation in the HFE gene that upregulates absorption or when it is ingested or infused at levels that exceed the body’s ability to clear it. Excess iron deposition in parenchymal tissue causes injury and ultimately organ dysfunction. Diabetes mellitus and hepatic cirrhosis due to pancreas and liver damage are just two examples of diseases that result from iron overload. Despite the rapid growth of information regarding iron metabolism and iron overload states, the most effective treatment is still serial phlebotomies. We present a patient who developed iron overload due to chronic ingestion of oral ferrous sulfate. This case illustrates the importance of querying geriatric patients regarding their use of nonprescription iron products without a medical indication. Ronald Lands and Emmanuel Isang Copyright © 2017 Ronald Lands and Emmanuel Isang. All rights reserved. Neurological Complications following Blood Transfusions in Sickle Cell Anemia Tue, 03 Jan 2017 08:31:49 +0000 http://www.hindawi.com/journals/crihem/2017/3649397/ In Sickle Cell Anemia (SCA) patient blood transfusions are an important part of treatment for stroke and its prevention. However, blood transfusions can also lead to complications such as Reversible Posterior Leukoencephalopathy Syndrome (RPLS). This brief report highlights two cases of SCA who developed such neurological complications after a blood transfusion. RLPS should be considered as the cause of neurologic finding in patients with SCA and hypertension following a blood transfusion. Hana Alharbi, Nayaab Khawar, Jolanta Kulpa, Anne Bellin, Simona Proteasa, and Revathy Sundaram Copyright © 2017 Hana Alharbi et al. All rights reserved. Spontaneous Remission in an Older Patient with Relapsed FLT3 ITD Mutant AML Thu, 29 Dec 2016 14:06:45 +0000 http://www.hindawi.com/journals/crihem/2016/1259759/ Spontaneous remission (SR) of acute myeloid leukemia (AML) is a very rare phenomenon. AML characterized by FLT3 internal tandem duplication (FLT3 ITD) is typically associated with an aggressive clinical course with rapid progression, relapse, and short overall survival in the absence of transplantation. We report here the first case of SR of FLT3 ITD mutant AML in the literature. Our patient was an elderly woman with relapsed NPM1 and FLT3 ITD mutant AML whose disease underwent SR for a brief duration without precipitating cause. We review the potential immune mechanisms underlying SR in AML and discuss the implications for novel immunotherapeutic approaches for FLT3 mutant AML. Pankit Vachhani, Jason H. Mendler, Andrew Evans, George Deeb, Petr Starostik, Paul K. Wallace, and Eunice S. Wang Copyright © 2016 Pankit Vachhani et al. All rights reserved. Hypercalcemia due to Primary Hepatic Lymphoma Sun, 25 Dec 2016 11:57:56 +0000 http://www.hindawi.com/journals/crihem/2016/1876901/ A 65-year-old female with a history of mixed connective tissue disease and pulmonary fibrosis on azathioprine, hydroxychloroquine, and prednisone (osteoporosis on teriparatide) presented with a 1-month history of hypercalcemia. After discontinuation of teriparatide, the patient’s hypercalcemia persisted. Further evaluation revealed primary hepatic lymphoma as the source of her hypercalcemia. Andrew Hsu, Michael Gagnier, Elizabeth Ryer, Mohammed Salhab, and Alan G. Rosmarin Copyright © 2016 Andrew Hsu et al. All rights reserved. Attempted Suicide by Massive Warfarin Ingestion Conservatively Managed Using Phytonadione Sun, 04 Dec 2016 14:26:47 +0000 http://www.hindawi.com/journals/crihem/2016/7095251/ Treatment strategies for acute toxicity following massive ingestion of warfarin are not well described in the literature. Warfarin is the primary oral anticoagulation agent used in the treatment of thromboembolic disease, and patients with acute toxicity are at risk for life-threatening hemorrhages. Treatment options include phytonadione (vitamin K1), fresh frozen plasma (FFP), and prothrombin complex concentrates (PCCs) used alone or in combination. FFP and PCC can be associated with volume complications, undesirable thromboembolic events, and increased costs. We describe the case of a 63-year-old female with acute warfarin toxicity following a massive ingestion of warfarin (420 mg–450 mg) in an attempt to commit suicide. Upon arrival to the emergency department, serial INR checks were initiated to help guide dosing strategy and later adjusted based on INR response to treatment using only phytonadione. Katherine L. March, Kruti S. Patel, and Jennifer D. Twilla Copyright © 2016 Katherine L. March et al. All rights reserved. Pseudothrombocytopenia due to Platelet Clumping: A Case Report and Brief Review of the Literature Sun, 04 Dec 2016 11:04:30 +0000 http://www.hindawi.com/journals/crihem/2016/3036476/ Platelet clumping is a common laboratory phenomenon that complicates or precludes reporting of platelet count. It is often, but not always, a phenomenon commonly caused by the anticoagulant EDTA. Herein, we discuss a case of a 14-year-old girl who was found to have platelet clumping and discuss the work-up she underwent to investigate her pseudothrombocytopenia. Geok Chin Tan, Melissa Stalling, Gretchen Dennis, Maria Nunez, and Samir B. Kahwash Copyright © 2016 Geok Chin Tan et al. All rights reserved. Minimally Invasive Diagnosis of Secondary Intracranial Lymphoma Mon, 28 Nov 2016 13:36:42 +0000 http://www.hindawi.com/journals/crihem/2016/6165172/ Diffuse large B cell lymphomas (DLBCL) are an aggressive group of non-Hodgkin lymphoid malignancies which have diverse presentation and can have high mortality. Central nervous system relapse is rare but has poor survival. We present the diagnosis of primary mandibular DLBCL and a unique minimally invasive diagnosis of secondary intracranial recurrence. This case highlights the manifold radiological contributions to the diagnosis and management of lymphoma. A. P. McClement, G. M. Healy, C. E. Redmond, E. Stocker, G. Connaghan, S. J. Skehan, and R. P. Killeen Copyright © 2016 A. P. McClement et al. All rights reserved. Coexistence of Factor VII Deficiency and Hereditary Spastic Paraplegia in Two Siblings Sun, 27 Nov 2016 12:30:24 +0000 http://www.hindawi.com/journals/crihem/2016/1351873/ We present the case of two patients aged 12 years and 7 years who were referred to our hospital for factor VII deficiency inherited in an autosomal recessive pattern, who had suffered from previous multiple joint haemarthroses. They presented with fine motor symptoms and difficulty in walking. During physical examination we observed neurological symptoms (general hypotonia, muscular hypotrophy, exaggerated tendon reflexes, pes cavus, and spastic gait). Given that the symptoms were not justified by the deficiency of coagulation factor VII and on suspicion of hereditary spastic paraplegia (HSP), tests were carried out. Findings from the tests confirmed the diagnosis of HSP (axonal degeneration of the central motor pathway and pyramidal tracts), further complicated by mixed neuropathy. This disease was also inherited in an autosomal recessive pattern with no direct genetic association with factor VII deficiency. Neurological symptoms had gone unnoticed due to a history of multiple joint haemarthrosis; musculoskeletal examination led to a satisfactory differential diagnosis. Haematological prophylaxis was commenced with rFVIIa at 30 mcg/kg, three days per week. A rehabilitation programme was prescribed so that the patient could remain independent for as long as possible, based on orthosis, physiotherapy, and occupational therapy. Response to treatment is currently satisfactory and no new bleeding has presented. As far as we are aware, the coexistence of these two diseases (factor VII deficiency and HSP) has not been previously reported in the literature. Hortensia De la Corte-Rodriguez, E. Carlos Rodriguez-Merchan, M. Teresa Alvarez-Roman, and Ana L. Hernandez-Moreno Copyright © 2016 Hortensia De la Corte-Rodriguez et al. All rights reserved. Ischemic Monomeric Neuropathy in a Woman with Sickle Cell Anaemia Tue, 22 Nov 2016 14:05:05 +0000 http://www.hindawi.com/journals/crihem/2016/8628425/ Sickle cell disease is an inherited haemoglobinopathy that can affect multiple organs and systems. The most common neurological complication in sickle cell disease is stroke and silent cerebral infarcts. Peripheral nervous system involvement has been described but is exceedingly rare. Herein, we describe the case of a young woman who presented with acute flaccid paralysis and sensory loss of the left lower extremity in the context of a painful vasoocclusive crisis which resolved rapidly after receiving an emergency automated red cell exchange transfusion. Alexandra Agapidou, Laura Aiken, Lisa Linpower, and Dimitris A. Tsitsikas Copyright © 2016 Alexandra Agapidou et al. All rights reserved. Total Body Irradiation without Chemotherapy as Conditioning for an Allogeneic Hematopoietic Cell Transplantation for Adult Acute Myeloid Leukemia Sun, 13 Nov 2016 09:49:22 +0000 http://www.hindawi.com/journals/crihem/2016/1257679/ Current therapies for acute myeloid leukemia (AML), failing induction, are rarely effective. We report our experience in 4 patients with AML who received 16 Gy TBI prior to allogeneic hematopoietic cell transplantation (alloHCT), between June 2010 and May 2011. Patients were 20 to 55 years of age, 2 with relapsed disease and 2 with AML failing induction. An HLA-matched graft from related or unrelated donor was infused on day 0. All but one, who received a CD34+-selected graft, received methotrexate and tacrolimus +/− antithymocyte globulin, as GVHD prophylaxis. The other patient received tacrolimus alone. Neutrophil and platelet engraftment occurred at a median of 18 and 14 days, respectively. Patients were discharged at a median of 28 days. There were no unexpected toxicities in the first 30 days. One patient had cytomegalovirus (CMV) viremia and anorexia, at two months. One patient had grade 2 acute GVHD of the skin. One patient developed chronic GVHD of the eyes, mouth, skin, joints, and lung at 4 months. Two patients died from relapse of their leukemia at days 65 and 125. Two patients remain in remission beyond day 1500. 16 Gy TBI followed by an alloHCT for AML, failing induction, is feasible and tolerable. Sultan Altouri, Mitchell Sabloff, David Allan, Harry Atkins, Lothar Huebsch, Dawn Maze, Rajiv Samant, and Christopher Bredeson Copyright © 2016 Sultan Altouri et al. All rights reserved. Bortezomib, Ifosfamide, Carboplatin, and Etoposide in a Patient with HIV-Negative Relapsed Plasmablastic Lymphoma Sun, 13 Nov 2016 08:09:59 +0000 http://www.hindawi.com/journals/crihem/2016/3598547/ Plasmablastic lymphoma (PBL) is a rare subtype of diffuse large B cell lymphoma (DLBCL), often associated with HIV infection. We present a case of a 53-year-old HIV-negative man with untreated hepatitis C viral infection who presented with abdominal pain and lymphadenopathy. Lymph node and bone marrow biopsies were consistent with plasmablastic lymphoma. He had partial response (PR) to 6 cycles of EPOCH but disease progressed seven weeks later. Repeat biopsy was consistent with plasmablastic lymphoma. Three cycles of bortezomib, ifosfamide, carboplatin, and etoposide (B-ICE) chemotherapy resulted in a partial response (PR). Five months later, he presented with widespread lymphadenopathy and tumor lysis syndrome with circulating blasts. Flow cytometry revealed a different population of lymphoma cells, this time positive for CD5, CD19, CD20, and CD22, with dim expression of CD45 and CD38. The patient died on the first day of ESHAP chemotherapy. There are no treatment recommendations or standard of care for plasmablastic lymphoma. A literature search yielded 10 cases in which bortezomib was administered in either HIV-positive or HIV-negative PBL. Six reported a partial response, 3 reported a complete response, and 1 was a near-complete response. Bortezomib, in combination with chemotherapy, may be an effective treatment option in PBL as reported here. Mehmet Akce, Elaine Chang, Mohammad Haeri, Mike Perez, Christie J. Finch, Mark M. Udden, and Martha P. Mims Copyright © 2016 Mehmet Akce et al. All rights reserved. Fludarabine Treatment of Patient with Chronic Lymphocytic Leukemia Induces a Digital Ischemia Thu, 03 Nov 2016 13:14:15 +0000 http://www.hindawi.com/journals/crihem/2016/7362791/ We report a 63-year-old man with a history of chronic lymphocytic leukemia (CLL) who presented with asymmetrical Raynaud’s phenomenon of sudden onset which progressed to acral gangrene rapidly in a week. These symptoms began approximately one week after the fourth cycle of fludarabine and cyclophosphamide chemotherapy and were accompanied by pain, numbness, and cyanosis in the fingers of his right hand except the first finger. Fludarabine may play a role in acral vascular syndrome. The treatment with fludarabine in patients with evolving digital ischemia should be carried out with caution. Utku Erdem Soyaltin, Deniz Yuce Yildirim, Mustafa Yildirim, Mehmet Can Ugur, Ferhat Ekinci, Cengiz Ceylan, and Harun Akar Copyright © 2016 Utku Erdem Soyaltin et al. All rights reserved.