Case Reports in Hematology http://www.hindawi.com The latest articles from Hindawi Publishing Corporation © 2016 , Hindawi Publishing Corporation . All rights reserved. A 26-Year-Old Female with Systemic Mastocytosis with Associated Myeloid Neoplasm with Eosinophilia and Abnormalities of PDGFRB, t(4;5)(q21;q33) Thu, 25 Aug 2016 13:33:43 +0000 http://www.hindawi.com/journals/crihem/2016/4158567/ Various translocations involving the PDGFRB gene are identified in myeloid neoplasms. However, the PRKG2/PDGFRB fusion gene associated with t(4;5)(q21;q33) has previously been reported in only 3 patients. We present the case of a 26-year-old woman with microcytic anemia, basophilia, thrombocytosis, and massive splenomegaly, who was found to have systemic mastocytosis and associated clonal hematological non-mast cell lineage disease (SM-AHNMD), with myeloid neoplasm with PRKG2/PDGFRB rearrangement. Initial findings included basophilia (37%, 4.1‚ÄČk/L), hypercellular marrow with eosinophilia, and increased and atypical megakaryocytes, suggestive of myeloproliferative neoplasm. Additional studies revealed large clusters of CD25 positive mast cells, fulfilling the criteria for the diagnosis of systemic mastocytosis. Consistent with prior reports of this translocation, our patient has responded well to imatinib. This case, in conjunction with others in the literature, suggests a possible connection between t(4;5)(q21;q33) PRKG2/PDGFRB and systemic mastocytosis and highlights their favorable response to imatinib. Laura E. Brown, Da Zhang, Diane L. Persons, Abdulraheem Yacoub, Shivani Ponnala, and Wei Cui Copyright © 2016 Laura E. Brown et al. All rights reserved. Long-Term Treatment with Romidepsin in Patients with Peripheral T-Cell Lymphoma Thu, 25 Aug 2016 13:07:30 +0000 http://www.hindawi.com/journals/crihem/2016/8175957/ Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of aggressive non-Hodgkin lymphomas. Angioimmunoblastic T-cell lymphoma (AITL) is a common subtype of PTCL, and patients with AITL typically have a poor prognosis with limited treatment options. Clinical studies have demonstrated the activity of romidepsin, a structurally unique, potent, bicyclic class 1 selective histone deacetylase inhibitor, in patients with relapsed or refractory AITL. In the case presented herein, we describe a patient treated with single-agent romidepsin at first diagnosis of AITL, resulting in complete remission for over 2 years and leading to the use of maintenance dosing. The patient eventually underwent a successful autologous stem cell transplant. This case illustrates the successful use of romidepsin for the long-term treatment of a patient with AITL in a clinical setting. Maintenance dosing may be an option for patients who have an extended response to romidepsin in order to optimize outcomes and to prolong time to the next subsequent line of therapy. In our case, the patient was able to remain in complete remission for more than 1 year while receiving maintenance dosing of romidepsin. Claudius Irlé and Jonathan Weintraub Copyright © 2016 Claudius Irlé and Jonathan Weintraub. All rights reserved. A Case of p63 Positive Diffuse Large B Cell Lymphoma of the Bladder Thu, 25 Aug 2016 13:01:49 +0000 http://www.hindawi.com/journals/crihem/2016/4348208/ Diffuse large B cell lymphoma (DLBCL), currently the most common type of non-Hodgkin lymphoma (NHL), is an aggressive B cell neoplasm that typically presents in older adults as a rapidly enlarging mass. The enlarging mass typically represents a lymph node, although extranodal disease can occur in a significant percentage (40%) of cases. The most common extranodal sites of involvement include the gastrointestinal tract and skin; primary bladder lymphoma represents only 0.2% of extranodal non-Hodgkin lymphomas. We report a case of diffuse large B cell lymphoma occurring in the bladder of an 83-year-old gentleman with an initial presentation of hematuria. This neoplasm displayed large, atypical cells with vesicular chromatin and prominent nucleoli that involved the bladder mucosa with invasion into muscularis propria, prostate, and urethra. Positive staining for p63 initially raised suspicion for poorly differentiated urothelial carcinoma; however, lack of staining for pancytokeratin and positive staining for LCA, CD20, CD79a, and PAX-5 confirmed the diagnosis of diffuse large B cell lymphoma. Though it does not occur in all cases, p63 can be positive in a significant percentage of cases of DLBCL; therefore, a diagnosis of lymphoma remains an important entity on the differential diagnosis of aggressive and particularly poorly differentiated neoplasms. Chelsey D. Deel, Carol Jones, and Teresa Scordino Copyright © 2016 Chelsey D. Deel et al. All rights reserved. Bacteremia due to Leuconostoc pseudomesenteroides in a Patient with Acute Lymphoblastic Leukemia: Case Report and Review of the Literature Mon, 22 Aug 2016 14:23:54 +0000 http://www.hindawi.com/journals/crihem/2016/7648628/ Leuconostoc species are vancomycin-resistant Gram-positive cocci. Infections due to Leuconostoc species have been reported in various immunocompromised patients, but little is known about such infection in patients with hematologic malignancies. We report a case of Leuconostoc infection in a 44-year-old woman with acute lymphoblastic leukemia. The patient developed a high fever despite antimicrobial therapy with doripenem after induction chemotherapy. After an isolate from blood cultures was identified as L. pseudomesenteroides, we changed the antibiotics to piperacillin-tazobactam and gentamicin, after which the patient recovered from the infection. Physicians should be aware of Leuconostoc species as causative pathogen if they encounter Gram-positive cocci bacteremia resistant to standard antibiotics such as vancomycin and teicoplanin, especially in patients with hematologic malignancies. Kazuko Ino, Kazunori Nakase, Kei Suzuki, Akiko Nakamura, Atsushi Fujieda, and Naoyuki Katayama Copyright © 2016 Kazuko Ino et al. All rights reserved. Acute Monocytic Leukemia Masquerading Behçet’s Disease-Like Illness at Onset in an Elderly Female Tue, 16 Aug 2016 16:07:00 +0000 http://www.hindawi.com/journals/crihem/2016/4231276/ A previously healthy 74-year-old Japanese female was hospitalized with fever and high C-reactive protein. She developed palatal herpangina-like aphthous ulcers, localized intestinal wall thickening, terminal ileum ulcers, and an erythematous acneiform rash; thus Behçet’s disease-like illness was suspected. Significant peripheral blood acute monocytosis developed during her hospitalization and acute monocytic leukemia (FAB M5b) with normal karyotype was diagnosed. By immunostaining, the infiltrating cells in the skin and the terminal ileum were identified as monocytic leukemic cells. This case exhibited a unique initial presentation of Behçet’s disease-like illness associated with acute monocytic leukemia. Shigeru Koba, Toshio Sekioka, Sorou Takeda, Aya Miyagawa-Hayashino, Keisuke Nishimura, and Shinsaku Imashuku Copyright © 2016 Shigeru Koba et al. All rights reserved. Simultaneous Manifestation of Chronic Myelomonocytic Leukemia and Multiple Myeloma during Treatment by Prednisolone and Eltrombopag for Immune-Mediated Thrombocytopenic Purpura Sun, 14 Aug 2016 06:10:54 +0000 http://www.hindawi.com/journals/crihem/2016/4342820/ An 80-year-old man was admitted to our hospital because of severe thrombocytopenia. He was diagnosed with idiopathic thrombocytopenia, and prednisolone together with eltrombopag was started, leading to significant improvement of platelet counts. Four years later, there was a prominent increase of peripheral blood monocytes, which was accompanied by recurrence of thrombocytopenia. Bone marrow aspirates and serum electrophoresis revealed coexistence of chronic myelomonocytic leukemia (CMML) and multiple myeloma (MM). The patient received lenalidomide plus dexamethasone therapy but died due to exacerbation of the disorder. It was supposed that thrombocytopenia was secondarily caused by CMML and MM developed at a later period. Masao Hagihara, Morihiro Inoue, Kenichiro Kodama, Tomoyuki Uchida, and Jian Hua Copyright © 2016 Masao Hagihara et al. All rights reserved. Case Report of Diffuse Large B Cell Lymphoma of Uterine Cervix Treated at a Semiurban Cancer Centre in North India Thu, 11 Aug 2016 16:14:14 +0000 http://www.hindawi.com/journals/crihem/2016/3042531/ Lymphoma of the uterine cervix is very rare. We report a case of diffuse large B cell lymphoma (DLBCL) involving the uterine cervix treated at a newly commissioned semiurban cancer centre in north India in 2015. Data for this study was obtained from the hospital electronic medical records and the patient’s case file. We also reviewed published case reports of uterine and cervical lymphoma involving forty-one patients. We treated a case of stage IV DLBCL cervix with six cycles of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) and intrathecal methotrexate followed by consolidation with radiotherapy. The patient showed complete response to chemotherapy. We conclude that, in advanced stage lymphoma involving uterus and cervix, combination of chemotherapy and radiotherapy is effective in short term. Vibhor Sharma, Tapas Dora, Mehul Patel, Sankalp Sancheti, and Epari Sridhar Copyright © 2016 Vibhor Sharma et al. All rights reserved. A Rare Cause of Hemophagocytic Lymphohistiocytosis: Fusobacterium Infection—A Case Report and Review of the Literature Wed, 03 Aug 2016 13:51:09 +0000 http://www.hindawi.com/journals/crihem/2016/4839146/ Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome characterized by excessive activation of the immune system. Bacterial infections are very rare precipitants of this disease. A 19-year-old gentleman presented with headache, fatigue, and malaise. He was found to be hypotensive, tachycardic, and febrile. Broad spectrum antibiotics were initiated, and a lumbar puncture ruled out meningitis. Patient progressively developed shock that required use of vasopressors, as well as renal and respiratory failure. Blood cultures grew Fusobacterium necrophorum. Given continued fevers despite appropriate antimicrobials, a bone marrow biopsy was performed revealing increased histiocytes with hemophagocytosis. Dexamethasone was added with dramatic clinical improvement. Our case highlights Fusobacterium as a rare precipitant of HLH and proves that a high index of clinical suspicion is crucial for early diagnosis of HLH, allowing for prompt initiation of HLH-specific immunosuppressive therapy that can be life-saving. Ghulam Rehman Mohyuddin and Heather J. Male Copyright © 2016 Ghulam Rehman Mohyuddin and Heather J. Male. All rights reserved. Steroid Refractory Autoimmune Haemolytic Anaemia Secondary to Sarcoidosis Successfully Treated with Rituximab and Mycophenolate Mofetil Tue, 02 Aug 2016 05:56:56 +0000 http://www.hindawi.com/journals/crihem/2016/9495761/ Autoimmune haemolytic anaemia is not a well-recognised complication of sarcoidosis. We describe the case of a 30-year-old female who presented with acute warm haemolytic anaemia and widespread lymphadenopathy. Sarcoidosis was diagnosed on lymph node biopsy and further investigation. The haemolytic anaemia responded only to a high dose of steroids. Evidence regarding treatment of steroid refractory autoimmune haemolysis secondary to sarcoidosis is lacking. Based on the emergent evidence that both disorders share common immunopathogenic mechanisms involving Th1 and Th17 lymphocytes, our patient was given rituximab and mycophenolate mofetil to successfully suppress the haemolysis and sarcoid activity. Sarah Green, Erica Partridge, Edore Idedevbo, and Anton Borg Copyright © 2016 Sarah Green et al. All rights reserved. Pernicious Anemia with Autoimmune Hemolytic Anemia: A Case Report and Literature Review Sun, 31 Jul 2016 07:22:16 +0000 http://www.hindawi.com/journals/crihem/2016/7231503/ Pernicious anemia is a common cause of vitamin B12 deficiency. Here, we discuss a case of a young woman who presented with severe anemia along with a history of iron deficiency anemia. After a review of her clinical presentation and laboratory data, we identified an autoimmune hemolytic anemia and a concomitant pernicious anemia. The concurrence of both these hematological diagnoses in a patient is rare. Sri Lakshmi Hyndavi Yeruva, Raj Pal Manchandani, and Patricia Oneal Copyright © 2016 Sri Lakshmi Hyndavi Yeruva et al. All rights reserved. Extranodal NK/T Cell Lymphoma Causing Cardiorespiratory Failure Sun, 17 Jul 2016 06:30:00 +0000 http://www.hindawi.com/journals/crihem/2016/2394809/ Extranodal NK/T cell lymphoma is an uncommon malignancy usually involving the sinonasal area. We report an unusual case of extranodal NK/T cell lymphoma diagnosed in a 62-year-old Caucasian male who died of progressive cardiorespiratory failure but had no clinically detectable upper respiratory system lesions. The initial diagnosis was made cytologically on a sample of pericardial fluid that contained neoplastic lymphoid cells. These cells were positive for CD2, cytoplasmic CD3, and Epstein-Barr virus and negative for CD56. The diagnosis was confirmed at the autopsy, which disclosed lymphoma infiltrates in the myocardium, lungs, stomach, and pancreas. The death was caused by heart and lung failure due to uncontrollable arrhythmia and respiratory insufficiency due to the lymphoma infiltrates. To the best of our knowledge, this is the first case of extranodal NK/T cell lymphoma presenting with cardiopulmonary failure. Yiting Li and Ivan Damjanov Copyright © 2016 Yiting Li and Ivan Damjanov. All rights reserved. Successful Control of Massive Bleeding in a Child with Burkitt’s Lymphoma via a Biosimilar Recombinant Activated Factor VII (AryoSeven™) Sun, 10 Jul 2016 14:02:53 +0000 http://www.hindawi.com/journals/crihem/2016/1295092/ We describe a case of a 4-year-old girl with Burkitt’s lymphoma, who suffered from a massive gastrointestinal hemorrhage 3 days after chemotherapy. In spite of applying the common practice in correction of coagulopathy, thrombocytopenia persisted and bleeding became life-threatening. In the present case report, we report a successful control of bleeding with a single-dose administration of a biosimilar recombinant activated human factor VII (AryoSeven). Kourosh Goudarzi Pour, Fatemeh Malek, and Peyman Eshghi Copyright © 2016 Kourosh Goudarzi Pour et al. All rights reserved. Successful Treatment and Secondary Prevention of Venous Thrombosis Secondary to Behçet Disease with Rivaroxaban Wed, 29 Jun 2016 12:08:59 +0000 http://www.hindawi.com/journals/crihem/2016/2164329/ We here present the successful initial treatment and secondary prophylaxis of superficial venous thrombosis secondary to Behçet’s disease by a novel anticoagulant drug, rivaroxaban (Xarelto®). To our knowledge, this is the first case of using an oral direct inhibitor of FXa in this setting. Our findings are promising; the outcome was favourable without any adverse effect noted. We propose that the patients with Behçet’s disease and venous thrombosis might benefit from the advantages of the new anticoagulant drug. Ana Boban, Catherine Lambert, and Cedric Hermans Copyright © 2016 Ana Boban et al. All rights reserved. A Case of Chyloperitoneum Secondary to Follicular Lymphoma and a Review of Prognostic Implications Sun, 26 Jun 2016 08:42:56 +0000 http://www.hindawi.com/journals/crihem/2016/4625819/ Chyloperitoneum, or chylous ascites, is a rare condition characterized by milky-appearing fluid with elevated triglyceride content and the presence of chylomicrons. Malignancy, specifically lymphoma, is reported to be the predominant cause in Western countries. Previously, the prognosis for patients with chyloperitoneum due to lymphoma has been reported as poor. We present a case of chyloperitoneum and chylothorax due to follicular lymphoma with excellent response to bendamustine and Rituxan. A review of the literature indicates that patients with chyloperitoneum associated with lymphoma generally have a favorable response to contemporary treatment regimens. Megan Jagosky, Brice Taylor, and Stephanie Parks Taylor Copyright © 2016 Megan Jagosky et al. All rights reserved. Wiskott-Aldrich Syndrome Presenting with JMML-Like Blood Picture and Normal Sized Platelets Tue, 31 May 2016 09:19:12 +0000 http://www.hindawi.com/journals/crihem/2016/8230786/ Objective. The aim of this paper is to report the case of Wiskott-Aldrich syndrome (WAS) that presented with unusual laboratory features. Clinical Presentation and Intervention. Male neonate admitted with symptoms related to thrombocytopenia, whose initial diagnosis was considered as neonatal alloimmune thrombocytopenia and JMML (juvenile myelomonocytic leukemia) but subsequently diagnosis was confirmed as WAS. Conclusion. This case shows that a suspicion of WAS is warranted in the setting of neonatal thrombocytopenia with JMML-like blood picture and normal sized platelets. Rajesh B. Patil, Chandrakala Shanmukhaiah, Farah Jijina, Shailesh Bamborde, Nilesh Wasekar, Manoj Toshniwal, Aniket Mohite, and Vinod Patil Copyright © 2016 Rajesh B. Patil et al. All rights reserved. Renal Thrombotic Microangiopathy Associated with the Use of Bortezomib in a Patient with Multiple Myeloma Mon, 16 May 2016 13:40:08 +0000 http://www.hindawi.com/journals/crihem/2016/6020691/ Bortezomib is a first-generation proteasome inhibitor used in the treatment of multiple myeloma (MM). A few reports have linked bortezomib exposure with the development of thrombotic microangiopathy (TMA). We describe a case of biopsy-proven renal thrombotic microangiopathy associated with the use of bortezomib in a 51-year-old man with IgG lambda MM. To our knowledge, this is the first biopsy-proven case. In addition, reexposure to bortezomib 18 months later was associated with recurrence of TMA. This supports a possible causal role of bortezomib. The exact mechanisms remain to be elucidated. Jan Van Keer, Michel Delforge, Daan Dierickx, Kathelijne Peerlinck, Evelyne Lerut, and Ben Sprangers Copyright © 2016 Jan Van Keer et al. All rights reserved. Paraneoplastic Syndrome in Splenic Marginal Zone Lymphoma: A Rare Phenomenon of Paraplegia as an Atypical Presenting Manifestation Wed, 11 May 2016 11:12:27 +0000 http://www.hindawi.com/journals/crihem/2016/7034167/ We describe a case presenting complaint of complete lower body paraparesis, which was discovered to have splenic marginal zone lymphoma (SMZL). While paraneoplastic syndromes are more common in tumors, such as small cell lung cancer, very few reports exist on this condition with SMZL. We describe such a rare entity with a clinical course spanning twenty-four months after diagnosis. Jessica Schering and Vijayalakshmi Donthireddy Copyright © 2016 Jessica Schering and Vijayalakshmi Donthireddy. All rights reserved. Primary MALT Lymphoma of the Breast Treated with Definitive Radiation Tue, 10 May 2016 09:48:03 +0000 http://www.hindawi.com/journals/crihem/2016/1831792/ We are reporting a case of a 59-year-old woman, with a family history of breast cancer, who presented with extranodal marginal zone lymphoma (MALT) of the left breast. She received definitive radiation therapy and remains without evidence of disease. Here, we present a case and review the current literature to determine the optimal treatment of this rare presentation of MALT. Mohammad Hissourou III, Sayyad Yaseen Zia, Mahfood Alqatari, James Strauchen, and Richard L. Bakst Copyright © 2016 Mohammad Hissourou III et al. All rights reserved. Bing-Neel Syndrome Case Report: A Previously Undocumented IgG Variant with MRI, PET/CT, and PET/MRI Imaging Wed, 06 Apr 2016 13:39:36 +0000 http://www.hindawi.com/journals/crihem/2016/3931709/ Waldenstrom’s macroglobulinaemia is the most commonly reported subtype of lymphoplasmacytic lymphoma (LPL); it is characterised by IgM secretion. Neurological complications are common usually as a result of hyperviscosity. In rare cases, cells can infiltrate the central nervous system; this is known as Bing-Neel syndrome. We report the case of a 57-year-old male with lymphoplasmacytic lymphoma of the IgG-subtype with neurological symptoms and the consequent finding of lymphoplasmacytoid cells in his cerebrospinal fluid as well as deposits on MRI and PET-CT imaging. This is the first report of Bing-Neel syndrome in IgG-subtype LPL. We discuss the biological and radiological markers of his disease, including PET imaging, which has been minimal in this area to date. Daniel Halperin, Simon Hallam, Athar Haroon, Tom Butler, and Samir Agrawal Copyright © 2016 Daniel Halperin et al. All rights reserved. Marked Improvement in Refractory TTP Directly after H. pylori Eradication Therapy Tue, 05 Apr 2016 11:54:00 +0000 http://www.hindawi.com/journals/crihem/2016/1568586/ Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder involving thrombotic microangiopathy and is characterized by increased platelet aggregation throughout the body. Acquired TTP can be triggered by a variety of conditions including infections. We hereby describe a case report of an 81-year-old female presenting to the internal medicine department with TTP and active chronic gastritis, positive for Helicobacter pylori (H. pylori) on biopsy. The TTP was highly resistant to medical therapy; however the patient underwent complete resolution of her TTP following H. pylori eradication. We conclude that acquired TTP may be triggered by H. pylori infection and that treating the underlying infection may play a role in improving TTP’s outcome in some patients, especially when disease is refractory to medical therapy. Irina Gringauz, Narin Nard Carmel-Neiderman, Tobin Mangel, Orith Portnoy, Gad Segal, and Idan Goren Copyright © 2016 Irina Gringauz et al. All rights reserved. Complete Remission of Acute Myeloid Leukemia following Cisplatin Based Concurrent Therapy with Radiation for Squamous Cell Laryngeal Cancer Sun, 03 Apr 2016 16:53:54 +0000 http://www.hindawi.com/journals/crihem/2016/8581421/ Acute myeloid leukemia (AML) is a myeloid disorder with several established treatment regimens depending on patient and leukemic factors. Cisplatin is known to have strong leukemogenic potential and is rarely used even as salvage therapy in relapsed or refractory AML. We present a patient simultaneously diagnosed with AML and squamous cell carcinoma of the larynx, who was found to be in complete remission from AML following treatment with cisplatin based chemoradiotherapy for his laryngeal cancer. Mayur D. Mody, Harpaul S. Gill, Kristin A. Higgins, Nabil F. Saba, and Vamsi K. Kota Copyright © 2016 Mayur D. Mody et al. All rights reserved. Pregnancy and Accelerated Phase of Myeloid Chronic Leukemia Treated with Imatinib: A Case Report from a Developing Country Thu, 31 Mar 2016 06:51:10 +0000 http://www.hindawi.com/journals/crihem/2016/6104948/ Background. Chronic myeloid leukemia is a hematological malignancy caused by expression of BCR-ABL tyrosine kinase oncogene, product of the t(9;22) Philadelphia translocation. Accelerated phase of this disease marks the onset of advanced rapidly progressive disease unresponsive to many therapies. Pregnancy limits broad number of therapies on patients because of their potential teratogenic effects. We report the case of a pregnant 34-year-old patient on accelerated phase successively managed by imatinib. She achieved a safe pregnancy and delivered at 39 weeks a healthy baby without congenital abnormalities. Our case is unusual because of the accelerated phase of the disease. Case Presentation. A 34-year-old African female with history of chronic phase of myeloid leukemia on imatinib, lost to follow-up for 4 months, presented to the hematological department for abdominal discomfort. Accelerated phase of chronic myeloid leukemia was diagnosed. Complete hematological response was achieved on high doses of imatinib. At the completion of 39 weeks, she delivered a healthy child without congenital anomalies. Conclusion. Despite its teratogenic and embryotoxic effects, front line imatinib is the only effective, well-tolerated treatment for patient on accelerated phase that can be offered to patients in sub-Saharan countries. Lydie Ocini Ngolet, Innocent Kocko, and Alexis Elira Dokekias Copyright © 2016 Lydie Ocini Ngolet et al. All rights reserved. Extra-Adrenal Myelolipoma Containing Small Lymphocytic Lymphoma/Chronic Lymphocytic Leukemia: A Case Report and Review of the Literature Tue, 29 Mar 2016 12:24:05 +0000 http://www.hindawi.com/journals/crihem/2016/7364951/ Myelolipoma is a benign tumor consisting of mature fat interspersed with hematopoietic elements resembling bone marrow. The vast majority occurs within the adrenal glands, but several cases of extra-adrenal myelolipomas (EAMLs) have been reported. We report a case of a 64-year-old male who presented with complaint of lower abdominal discomfort. CT scan of abdomen and pelvis showed a 6 cm × 5 cm, well-circumscribed, predominantly fatty mass in the presacral region. Histological examination of the pelvic mass revealed a myelolipoma heavily infiltrated by small lymphoid cell aggregates with immunophenotypic features of small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL). Review of the literature revealed that there is only one published report of SLL/CLL involving a myelolipoma, which was also an extra-adrenal myelolipoma, and, therefore, our case is the second case of a SLL/CLL involving a myelolipoma that is an extra-adrenal myelolipoma. Extra-adrenal myelolipomas seem to the preferred myelolipomas for involvement by SLL/CLL. Komal Arora and Jagmohan Sidhu Copyright © 2016 Komal Arora and Jagmohan Sidhu. All rights reserved. Refractory IgG Warm Autoimmune Hemolytic Anemia Treated with Eculizumab: A Novel Application of Anticomplement Therapy Tue, 22 Mar 2016 11:52:10 +0000 http://www.hindawi.com/journals/crihem/2016/9181698/ Warm autoimmune hemolytic anemia (wAIHA) is the most common form of AIHA, with corticosteroids in first-line treatment resulting in a 60–80% response rate. Atypical wAIHA and IgG plus complement mediated disease have a higher treatment failure rate and higher recurrence rate. We report a case of severe wAIHA secondary to Waldenström macroglobulinemia with life threatening intravascular hemolysis refractory to prednisone, rituximab, splenectomy, and plasmapheresis. A four-week treatment of eculizumab in this heavily pretreated patient resulted in a sustained increase in hemoglobin and transfusion independence, suggesting a role for complement inhibition in refractory wAIHA. Kim Ma and Stephen Caplan Copyright © 2016 Kim Ma and Stephen Caplan. All rights reserved. Pure White Cell Aplasia and Necrotizing Myositis Thu, 17 Mar 2016 11:58:41 +0000 http://www.hindawi.com/journals/crihem/2016/4161679/ Pure white cell aplasia (PWCA) is a rare hematologic disorder characterized by the absence of neutrophil lineages in the bone marrow with intact megakaryopoiesis and erythropoiesis. PWCA has been associated with autoimmune, drug-induced, and viral exposures. Here, we report a case of a 74-year-old female who presented with severe proximal weakness without pain and was found to have PWCA with nonspecific inflammatory necrotizing myositis and acute liver injury on biopsies. These findings were associated with a recent course of azithromycin and her daily use of a statin. Myositis improved on prednisone but PWCA persisted. With intravenous immunoglobulin and granulocyte-colony stimulating factor therapies, her symptoms and neutrophil counts improved and were sustained for months. Peter Geon Kim, Joome Suh, Max W. Adelman, Kwadwo Oduro, Erik Williams, Andrew M. Brunner, and David J. Kuter Copyright © 2016 Peter Geon Kim et al. All rights reserved. Cytogenetically Unrelated Clones in Acute Myeloid Leukemia Showing Different Responses to Chemotherapy Thu, 10 Mar 2016 08:40:54 +0000 http://www.hindawi.com/journals/crihem/2016/2373902/ We report a case of acute myeloid leukemia (AML) with two cytogenetically unrelated clones. The patient was a 45-year-old male who was diagnosed with acute monoblastic leukemia (AMoL). Initial G-band analysis showed 51,XY,+6,+8,inv(9)(p12q13)c,+11,+13,+19[12]/52,idem,+Y[8], but G-band analysis after induction therapy showed 45,XY,-7,inv(9)(p12q13)c[19]/46,XY,inv(9)(p12q13)c[1]. Retrospective FISH analysis revealed a cryptic monosomy 7 clone in the initial AML sample. The clone with multiple trisomies was eliminated after induction therapy and never recurred, but a clone with monosomy 7 was still detected in myelodysplastic marrow with a normal blast percentage. Both clones were successfully eliminated after related peripheral blood stem cell transplantation, but the patient died of relapsed AML with monosomy 7. We concluded that one clone was de novo AMoL with chromosome 6, 8, 11, 13, and 19 trisomy and that the other was acute myeloid leukemia with myelodysplasia-related changes (AML-MRC) with chromosome 7 monosomy showing different responses to chemotherapy. Simultaneous onset of cytogenetically unrelated hematological malignancies that each have a different disease status is a rare phenomenon but is important to diagnose for a correct understanding of the disease status and for establishing an appropriate treatment strategy. Kohei Kasahara, Masahiro Onozawa, Naohiro Miyashita, Emi Yokohata, Miho Yoshida, Minoru Kanaya, Mizuha Kosugi-Kanaya, Ryo Takemura, Shojiro Takahashi, Junichi Sugita, Akio Shigematsu, Mutsumi Takahata, Shinichi Fujisawa, Daigo Hashimoto, Katsuya Fujimoto, Tomoyuki Endo, Takeshi Kondo, and Takanori Teshima Copyright © 2016 Kohei Kasahara et al. All rights reserved. Simeprevir and Sofosbuvir Combination Treatment in a Patient with HCV Cirrhosis and HbS Beta 0-Thalassemia: Efficacy and Safety despite Baseline Hyperbilirubinemia Wed, 02 Mar 2016 06:08:57 +0000 http://www.hindawi.com/journals/crihem/2016/7635128/ Hyperbilirubinemia is an adverse reaction of simeprevir (SMV). The majority of these patients were taking concurrent ribavirin presenting elevated unconjugated hyperbilirubinemia due to hemolysis. However, cases of hepatic failure with elevated bilirubin level have also been reported in patients with decompensated cirrhosis. We describe a 51-year-old female patient with HbS beta 0-thalassemia and recently diagnosed compensated cirrhosis due to chronic hepatitis C infection. Laboratory evaluation revealed total bilirubin: 2.7 mg/dL and serum HCV-RNA 137.204 IU/mL. HCV was genotyped as 4. A FibroScan revealed 35.3 kPa. She was considered as illegible for pegylated-interferon-free treatment with direct acting antivirals and a course with simeprevir and sofosbuvir (SOF) combination for twelve weeks was planned. Hyperbilirubinemia developed from the beginning with peak values during the 3rd month of treatment. However, no findings of liver decompensation were noticed. Hyperbilirubinemia was benign and fully reversible and our patient finally achieved sustained virological response 24 weeks after the end of treatment. Nikolaos Papadopoulos, Melanie Deutsch, Athanasios Georgalas, Helias Poulakidas, and Lazaros Karnesis Copyright © 2016 Nikolaos Papadopoulos et al. All rights reserved. Erythrocytosis due to PHD2 Mutations: A Review of Clinical Presentation, Diagnosis, and Genetics Mon, 29 Feb 2016 11:10:45 +0000 http://www.hindawi.com/journals/crihem/2016/6373706/ The association of mutations in the PHD2 protein of the hypoxia-sensing pathway and erythrocytosis has only been established in the last decade. Here we report the case of a novel PHD2 gene mutation in a patient with erythrocytosis and summarize all reported cases to date. Case Report. A 55-year-old man presented with dyspnea and a previous diagnosis of idiopathic erythrocytosis. PHD gene sequencing revealed a mutation on exon 2. The mutation was recognized as p.(Trp) (c. 1001G>A) resulting in a truncation of a highly conserved amino acid residue in catalytic domain. A diagnosis of erythrocytosis secondary to mutant PHD2 gene was made. Conclusions. Our findings indicate that with PHD2 mutations there is moderate erythrocytosis and erythropoietin (Epo) levels are generally low to normal. Two patients with PHD2 substitution mutations were found to have paraganglioma and one of these patients had a concurrent pheochromocytoma. In addition, one mutation was associated with sagittal sinus thrombosis. Given the severity of some of the clinical features of these mutations, we conclude that clinical guidelines should include the PHD2 mutation in the idiopathic erythrocytosis workup. Rachel Wilson, Nausheen Syed, and Prabodh Shah Copyright © 2016 Rachel Wilson et al. All rights reserved. An Unusual Presentation of Pseudothrombotic Microangiopathy in a Patient with Autoimmune Atrophic Gastritis Thu, 25 Feb 2016 08:29:02 +0000 http://www.hindawi.com/journals/crihem/2016/1087831/ Introduction. We hereby describe the case of a young female patient who presented with pseudothrombotic microangiopathy, as well as pancytopenia accompanied by autoimmune atrophic gastritis. Case Presentation. A 36-year-old Caucasian woman presented to the emergency department with fatigue and dyspnea on minimal exertion. Physical examination was unremarkable except for pallor and noninjected conjunctiva. Laboratory tests revealed high LDH and low hemoglobin, white blood cells, platelets, and haptoglobin. The peripheral blood smear showed schistocytes suggestive of pseudothrombotic microangiopathy. Low cobalamin level and hyperhomocysteinemia were also detected. Autoimmune atrophic gastritis was confirmed by gastric biopsy and positive anti-intrinsic factor antibodies. Vitamin B12 supplements were given which led to rapid recovery and normalization of blood parameters. Conclusion. This case highlights the importance and serves as a reminder to clinicians to rule out cobalamin deficiency and autoimmune atrophic gastritis in patients presenting with a picture suggestive of thrombotic thrombocytopenic purpura and pancytopenia, which was completely reversible after appropriate replacement therapy without recurring to unnecessary and invasive procedures such as plasma exchange. Alexandre Malek and Roy Nasnas Copyright © 2016 Alexandre Malek and Roy Nasnas. All rights reserved. Postinfluenza Vaccination Idiopathic Thrombocytopenic Purpura in Three Elderly Patients Sun, 21 Feb 2016 11:33:33 +0000 http://www.hindawi.com/journals/crihem/2016/7913092/ The etiologies of secondary idiopathic thrombocytopenic purpura (ITP) include infection, autoimmune disease, and immunodeficiency. We report the cases of three elderly patients who developed ITP after receiving influenza vaccinations. The platelet count of an 81-year-old woman fell to 27,000/μL after she received an influenza vaccination. A 75-year-old woman developed thrombocytopenia (5,000 platelets/μL) after receiving an influenza vaccination. An 87-year-old woman whose laboratory test values included a platelet count of 2,000/μL experienced genital bleeding after receiving an influenza vaccination. After Helicobacter pylori (HP) eradication or corticosteroid treatment, all of the patients’ platelet counts increased. Influenza vaccination is an underlying etiology of ITP in elderly patients. HP eradication or corticosteroid treatment is effective for these patients. Clinicians should be aware of the association between ITP and influenza vaccinations. Joji Nagasaki, Masahiro Manabe, Kentaro Ido, Hiroyoshi Ichihara, Yasutaka Aoyama, Tadanobu Ohta, Yoshio Furukawa, and Atsuko Mugitani Copyright © 2016 Joji Nagasaki et al. All rights reserved.