Case Reports in Hematology https://www.hindawi.com The latest articles from Hindawi © 2018 , Hindawi Limited . All rights reserved. Antiphospholipid Antibody Induced by Nivolumab Thu, 11 Jan 2018 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2018/3106852/ Nivolumab is a monoclonal antibody against the programmed death protein 1 and is used for patients with advanced melanoma. It is associated with potentially immune-related adverse events, including disorders of the skin, GI tract, and the thyroid; these disorders were successfully treated with prednisone and infliximab. Other immunotherapeutic agents were observed to induce the formation of antiphospholipid antibody (APA) including α-interferon and interleukin-2. We present a case of APA development after the third dose of nivolumab in a 71-year-old male with advanced melanoma. The APA was detected after finding a prolonged aPTT; the lupus anticoagulant assay tested positive. The patient was treated with prednisone but, unfortunately, he expired a few days later. Ahmed Aburahma, Nour Aljariri Alhesan, Farah Elounais, and Emad Abu Sitta Copyright © 2018 Ahmed Aburahma et al. All rights reserved. Iatrogenic T-Cell Lymphoma with Associated Hemophagocytic Lymphohistiocyotsis in a Patient with Long-Standing Rheumatoid Arthritis Thu, 11 Jan 2018 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2018/8097965/ Patients with rheumatoid arthritis are at increased risk of hematological malignancies, especially when exposed to immunosuppressive therapy. The mechanisms of lymphomagenesis remain poorly understood but factors implicated include high disease activity, exposure to antitumoral necrosis factor medications, and Epstein–Barr virus infection. Lymphoid malignancies of T-cell origin are uncommon in patients with rheumatoid arthirits. Clinical presentation with associated hemophagocytic lymphohistiocyotsis is rare and confers a poor prognosis. This case report illustrates a case of a patient with long-standing rheumatoid arthritis and an iatrogenic peripheral T-cell lymphoma with secondary hemophagocytic lymphohistiocytosis who achieved a complete response after intensive chemotherapy. X. A. Andrade, H. E. Fuentes, D. M. Oramas, H. Mann, and P. Kovarik Copyright © 2018 X. A. Andrade et al. All rights reserved. Refractory Abdominal Pain in a Patient with Chronic Lymphocytic Leukemia: Be Wary of Acquired Angioedema due to C1 Esterase Inhibitor Deficiency Wed, 10 Jan 2018 09:37:57 +0000 http://www.hindawi.com/journals/crihem/2018/7809535/ Acquired angioedema due to C1 inhibitor deficiency (C1INH-AAE) is a rare and potentially fatal syndrome of bradykinin-mediated angioedema characterized by episodes of angioedema without urticaria. It typically manifests with nonpitting edema of the skin and edema in the gastrointestinal (GI) tract mucosa or upper airway. Edema of the upper airway and tongue may lead to life-threatening asphyxiation. C1INH-AAE is typically under-diagnosed because of its rarity and its propensity to mimic more common abdominal conditions and allergic reactions. In this article, we present the case of a 62-year-old male with a history of recently diagnosed chronic lymphocytic leukemia (CLL) who presented to our hospital with recurrent abdominal pain, initially suspected to have Clostridium difficile colitis and diverticulitis. He received a final diagnosis of acquired angioedema due to C1 esterase inhibitor deficiency due to concomitant symptoms of lip swelling, cutaneous nonpitting edema of his lower extremities, and complement level deficiencies. He received acute treatment with C1 esterase replacement and icatibant and was maintained on C1 esterase infusions. He also underwent chemotherapy for his underlying CLL and did not experience further recurrence of his angioedema. Abdullateef Abdulkareem, Ryan S. D’Souza, Joshua Mundorff, Pragya Shrestha, Oluwaseun Shogbesan, and Anthony Donato Copyright © 2018 Abdullateef Abdulkareem et al. All rights reserved. Acquired Coagulopathy and Hemorrhage Secondary to Subcutaneous Heparin Prophylaxis Wed, 10 Jan 2018 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2018/9501863/ Unfractionated heparin and low-molecular-weight heparins are commonly used as thromboprophylaxis for hospitalized patients. Though generally considered safe at prophylactic doses, cases of catastrophic hemorrhage have been reported. The proposed mechanism involves bioaccumulation of heparin through saturation of the rapid-elimination pathway in its metabolism. We present an unusual case of an average-weight man with metastatic melanoma who suffered hemorrhage with syncope and end-organ damage while on prophylactic three times daily unfractionated heparin. Coagulation studies were consistent with heparin toxicity. Despite administration of protamine, the clearance of heparin was remarkably delayed, as demonstrated by serial coagulation studies. We review the suspected risk factors for heparin bioaccumulation and the emerging understanding of this unusual adverse event involving a nearly ubiquitous medication. Maria Sunseri, Tania Ahuja, Tanya Wilcox, and David Green Copyright © 2018 Maria Sunseri et al. All rights reserved. Agranulocytosis Associated with Topiramate: A Case Report and Review of Published Cases Wed, 10 Jan 2018 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2018/5846398/ A 41-year-old female presented to the hospital with sore throat and shortness of breath. She was hypoxic with an oxygen saturation of 87% in room air. Physical examination revealed swollen uvula with exudates. She had been started on topiramate for treatment of migraine few months ago. The dose of topiramate was increased to 100 mg twice daily 2 weeks ago. Complete blood count revealed an absolute neutrophil count (ANC) of 8 c/mm3. She was intubated and started on broad-spectrum antibiotics. She was transferred to our hospital on the fifth day of hospitalization. On arrival, her absolute neutrophil count was 10 c/mm3. Her agranulocytosis was attributed to topiramate after ruling out other possible causes. ANC improved after topiramate was stopped. ANC increased to 1000 after 5 days of stopping topiramate. We also reviewed published cases of topiramate-associated agranulocytosis. Agranulocytosis is a rare side effect of topiramate, and only 3 case reports have been published so far. In all cases, agranulocytosis developed after months of topiramate therapy and when dose was increased to 200 mg daily suggesting a dose-dependent effect. Next steps would be further research on the pathogenesis of agranulocytosis associated with topiramate and creation of registry for data synthesis. Saroj Lohani, Niranjan Tachamo, Salik Nazir, and Anthony Donato Copyright © 2018 Saroj Lohani et al. All rights reserved. Morphological Transformation of Myeloma Cells into Multilobated Plasma Cell Nuclei within 7 Days in a Case of Secondary Plasma Cell Leukemia That Finally Transformed as Anaplastic Myeloma Thu, 21 Dec 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/5758368/ A 48-year-old man was diagnosed with multiple myeloma (IgG-k) and was treated with high-dose dexamethasone as an induction therapy followed by thalidomide-based regimens. Approximately 22 months after the initial diagnosis, the patient developed secondary plasma cell leukemia (PCL) with a white blood cell (WBC) count of 20.2 × 109/L including 79.5% plasma cells. A G-banding chromosomal analysis in the bone marrow showed an t(11;14) abnormality of up to 5%, which was not detected at initial diagnosis. We immediately started bortezomib and dexamethasone therapy, but in just 7 days, the WBC count elevated to 48.5 × 109/L, and approximately 95% of them were medium-sized atypical lymphoid cells with multilobated nuclei. Although we subsequently initiated alternative regimens, the patient’s condition deteriorated, and he died 4 months after developing PCL. Approximately 2 months before his death, the diameter of myeloma cells in the bone marrow enlarged by approximately twofold, and pleomorphic nuclei were present, indicating an anaplastic myeloma transformation. Concurrently, a 100% increase of the t(11;14) clone frequency was observed in the G-banding-analyzed bone marrow cells. Morphological transformation of myeloma cells into multilobated plasma cell nuclei can be considered as the starting point of the sequential process leading to anaplastic myeloma. Akihito Fujimi, Yasuhiro Nagamachi, Naofumi Yamauchi, and Yuji Kanisawa Copyright © 2017 Akihito Fujimi et al. All rights reserved. Sequential Use of Second-Generation Tyrosine Kinase Inhibitor Treatment and Intensive Chemotherapy Induced Long-Term Complete Molecular Response in Imatinib-Resistant CML Patient Presenting as a Myeloid Blast Crisis Sun, 17 Dec 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3209305/ Myeloid blast crisis of chronic myeloid leukemia (CML-MBC) is rarely seen at presentation and has a poor prognosis. There is no standard therapy for CML-MBC. It is often difficult to distinguish CML-MBC from acute myeloid leukemia expressing the Philadelphia chromosome (Ph+ AML). We present a case in which CML-MBC was seen at the initial presentation in a 75-year-old male. He was treated with conventional AML-directed chemotherapy followed by imatinib mesylate monotherapy, which failed to induce response. However, he achieved long-term complete molecular response after combination therapy involving dasatinib, a second-generation tyrosine kinase inhibitor, and conventional chemotherapy. Masaaki Tsuji, Tatsuki Uchiyama, Chisaki Mizumoto, Tomoharu Takeoka, Kenjiro Tomo, and Tatsuharu Ohno Copyright © 2017 Masaaki Tsuji et al. All rights reserved. Sickle Cell Beta-Plus Thalassemia with Subcapsular Hematoma of the Spleen Thu, 14 Dec 2017 09:15:58 +0000 http://www.hindawi.com/journals/crihem/2017/3819457/ While splenic complications like hypersplenism, sequestration crisis, and infarction are commonly reported in sickle cell variants like sickle cell beta-plus thalassemia, splenic rupture with hematoma is rare. We present a case of a 32-year-old young male who presented with dull left upper quadrant pain who was found to have multiple subcapsular splenic lacerations and hematoma on abdominal imaging. Hemoglobin electrophoresis confirmed sickle cell beta-plus thalassemia in the patient. There was no history of trauma, and rest of the workup for possible cause of spontaneous rupture of spleen was negative. With the patient refusing splenectomy, he was managed conservatively. Clinicians need to be aware of this rare complication of sickle cell variants. Suyash Dahal, Sumit Dahal, Dipesh K. C. Ghimire, Ebad Ur Rahman, and Eliza Sharma Copyright © 2017 Suyash Dahal et al. All rights reserved. Late Emergence of an Imatinib-Resistant ABL1 Kinase Domain Mutation in a Patient with Chronic Myeloid Leukemia Mon, 11 Dec 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3548936/ The introduction of the tyrosine kinase inhibitor (TKI) imatinib has revolutionised the outlook of chronic myeloid leukemia (CML); however, a significant proportion of patients develop resistance through several mechanisms, of which acquisition of ABL1 kinase domain mutations is prevalent. In chronic-phase patients, these mutations become evident early in the disease course. A case is described of a chronic-phase CML patient who achieved a sustained, deep molecular response but who developed an Y253H ABL1 kinase domain mutation nearly nine years after commencing imatinib. Switching therapy to bosutinib resulted in a rapid reachievement of a major molecular response. Long-term TKI treatment impacts on quality of life and late losses of responses are usually due to lack of adherence. This case highlights the requirement for ABL1 kinase domain mutation analysis in those CML patients on long-term imatinib who lost their molecular response, regardless of whether nonadherence is suspected. Mireille Crampe, Claire Andrews, Anne Fortune, and Stephen E. Langabeer Copyright © 2017 Mireille Crampe et al. All rights reserved. Peripheral T-Cell Lymphoma of the Submandibular Salivary Gland as an Unusual Manifestation of Richter’s Syndrome: A Case Report and Literature Review Sun, 03 Dec 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/1262368/ Richter’s syndrome is the development of high-grade non-Hodgkin lymphoma (NHL) or Hodgkin lymphoma in patients with chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL). In most patients with Richter’s syndrome, the high-grade NHL is diffuse large B-cell lymphoma. Only a small minority of CLL/SLL patients develop T-cell malignancies. Herein, we describe a 40-year-old male patient presenting with peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) in the submandibular salivary gland, two years after the diagnosis of CLL/SLL. The PTCL-NOS consisted of small lymphocytes, which complicated diagnosis. Immunohistochemical, cytological, and molecular studies allowed the correct diagnosis of composite lymphoma (SLL/PTCL-NOS) of the submandibular salivary gland. The PTCL-NOS had a cytotoxic phenotype and aberrant expression of CD79a. There was no evidence to suggest that the PTCL-NOS of the submandibular salivary gland developed from an intimately associated submandibular lymph node or by PTCL-NOS dissemination. A review of the literature and presented case suppose that the PTCLs developed following CLL/SLL have the cytotoxic phenotype and can clinically mimic typical Richter’s syndrome. Vadim R. Gorodetskiy, Natalya A. Probatova, and Tatiana T. Kondratieva Copyright © 2017 Vadim R. Gorodetskiy et al. All rights reserved. Plasmablastic Lymphoma with Coexistence of Chronic Lymphocytic Leukemia in an Immunocompetent Patient: A Case Report and Mini-Review Mon, 20 Nov 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/2861596/ Background. Plasmablastic lymphoma (PBL) is a rare, aggressive B-cell lymphoma with poor prognosis usually found in the oral cavity of HIV-positive patients. Chronic lymphocytic leukemia (CLL) is an indolent B-cell lymphoma with a variable clinical course. Transformation of CLL to PBL as Richter’s syndrome is rare while coexistence of CLL and PBL at diagnosis is even rarer. Case Report. We describe a case of a male immunocompetent patient with an ileum-cecum valve mass and a soft tissue mass at the left humerus with histologic evidence of PBL with coexistence of CLL in the bone marrow and peripheral blood. Amputation of the patient’s left arm was inevitable, and the patient was started on bortezomib and dexamethasone. However, prolonged hospitalization was complicated by aspiration pneumonia, and the patient passed away. Conclusions. No standard of care exists for patients with PBL, and prognosis remains dismal. Concomitant presentation of hematological malignancies becomes increasingly recognized, and further insight is needed in order to delineate whether they originate from the same clone or from different ones. Eleftheria Hatzimichael, Konstantina Papathanasiou, Ioannis Zerdes, Stefanos Flindris, Alexandra Papoudou-Bai, and Eleni Kapsali Copyright © 2017 Eleftheria Hatzimichael et al. All rights reserved. Waldenstrom’s Macroglobulinemia: A Report of Two Cases, One with Severe Retinopathy and One with Renal Failure Tue, 31 Oct 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3732902/ We report here two cases of Waldenstrom’s macroglobulinemia (WM), one with central nervous system (CNS) symptoms and severe retinopathy and one with renal failure. In both cases, the serum IgM levels exceeded 3,000 mg/dL and monoclonal IgM-kappa was observed in the blood. At onset, Case 1, a 63-year-old female, developed CNS symptoms—namely, drowsiness and syncope. Case 2, a 58-year-old male, had nausea and dysgeusia on admission associated with renal failure, which is quite rare in patients with WM. Both patients exhibited hyperviscosity-related retinopathy, but it was particularly severe in Case 1: she suddenly lost her vision after admission. However, her vision recovered completely during treatment. Case 2 required hemodialysis immediately after admission. Needle biopsy of his kidney revealed tubulointerstitial nephritis with marked infiltration with CD20-positive lymphoplasmacytic lymphoma cells. After treatment, Case 1 has been in a remission longer than 8 years, but Case 2 died of pneumonia in 6 months. Since the initial symptoms of WM are ambiguous and vary significantly and hyperviscosity-related ophthalmological problems or severe renal dysfunction can arise, it is essential to promptly measure serum IgM levels and to institute appropriate care immediately when WM is confirmed in a patient. Naoko Kudo, Masakatsu Usui, Yukiharu Nakabo, Ken-ichi Yoshida, Kenji Miki, Takashi Osafune, Keisuke Nishimura, and Shinsaku Imashuku Copyright © 2017 Naoko Kudo et al. All rights reserved. Erdheim-Chester Disease with No Skeletal Bone Involvement and Massive Weight Loss Mon, 30 Oct 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3862052/ Erdheim-Chester disease (ECD) is a rare type of non-Langerhans cell histiocytosis, with only 550 cases reported worldwide. ECD is characterized by diffuse histiocytic infiltration of multiorgans. The age of presentation of this disease is typically between 40 and 70 years. Bone disease is the most common symptom, as unique radiological findings of long bone sclerosis occur in 96% of cases. Furthermore, BRAF V600E mutation is detected in 60% of ECD cases. In this manuscript, we are describing a unique case of ECD; the patient is younger than most reported cases and has no bone pain or any skeletal involvement. This patient has unintentionally lost about 50% of his body mass and is suffering from progressive cerebellar manifestations with radiological evidence of cerebellar atrophy, in contrast to the usual ECD manifestation of cerebellar infiltration. In addition, the patient has cardiac, retroperitoneal, and perinephric involvement, but he retains his sexual drive and fertility. A tissue biopsy from the retroperitoneal mass displayed typical morphological and immunohistochemical features of ECD, and BRAF V600E mutation was detected. He was treated with pegylated interferon alpha, but his disease progressed and the treatment was changed to vemurafenib to which he had an excellent response at 6 weeks. Hind Salama, Suleiman Kojan, Shaima Abdulrahman, Fahad Azzumeea, and Ayman Alhejazi Copyright © 2017 Hind Salama et al. All rights reserved. Severe Cytomegalovirus Reactivation in Patient with Low-Grade Non-Hodgkin’s Lymphoma after Standard Chemotherapy Sun, 22 Oct 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/5762525/ Clinically significant cytomegalovirus (CMV) reactivation is not uncommon in patients with severe immunodeficiency secondary to underlying medical disorders or following aggressive immunosuppressive therapy. However, it is less frequently found in patients with low-grade haematological malignancies after nonintensive chemotherapy. We treated a patient at our centre for stage IVB follicular lymphoma with standard chemotherapy who successively developed CMV colitis associated with a CMV viral load of >3 million copies/ml. Four lines of antiviral treatment were necessary to obtain biochemical remission with undetectable CMV levels, with an initially insufficient response to valganciclovir despite therapeutic pre- and posttreatment levels. Subsequently, our patient also developed an infection with Pneumocystis jirovecii pneumonia (PJP) as further evidence of severe immune compromise. This case report demonstrates the importance of including investigations for less common sources of infection when confronted with a patient with a low-grade haematological malignancy and a pyrexia of unknown origin. Lena Modvig, Ciaran Boyle, Katie Randall, and Anton Borg Copyright © 2017 Lena Modvig et al. All rights reserved. Chronic Myeloid Leukemia with an e6a2 BCR-ABL1 Fusion Transcript: Cooperating Mutations at Blast Crisis and Molecular Monitoring Mon, 16 Oct 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/9071702/ A minority of chronic myeloid leukemia patients (CML) express a variety of atypical BCR-ABL1 fusion variants and, of these, the e6a2 BCR-ABL1 fusion is generally associated with an aggressive disease course. Progression of CML to blast crisis is associated with acquisition of additional somatic mutations yet these events have not been elucidated in patients with the e6a2 BCR-ABL1 genotype. Moreover, molecular monitoring is only sporadically performed in CML patients with atypical BCR-ABL1 fusion transcripts due to lack of consensus approaches or standardization. A case of CML is described in which comprehensive molecular analysis, including targeted next-generation sequencing, revealed a single ASXL1 mutation cooperating with an e6a2 BCR-ABL1 fusion transcript at blast crisis. A quantitative molecular monitoring approach was devised and adopted that reflected the disease response from initial treatment through allogeneic stem cell transplantation which resulted in undetectable e6a2 BCR-ABL1 transcripts. This case emphasizes the requirement for molecular monitoring in CML patients with atypical BCR-ABL1 fusion transcripts and emphasizes that comprehensive sequencing has the potential to identify targets for novel therapies in CML patients with advanced disease. Mireille Crampe, Karl Haslam, Emma Groarke, Eileen Kelleher, Derville O’Shea, Eibhlin Conneally, and Stephen E. Langabeer Copyright © 2017 Mireille Crampe et al. All rights reserved. CD56-Negative Aggressive NK Cell Leukemia Relapsing as Multiple Cranial Nerve Palsies: Case Report and Literature Review Sun, 15 Oct 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3724017/ Background. Aggressive natural killer cell leukemia (ANKL) is extremely rare and habitually manifests as a systemic disease with multiorgan failure that rapidly evolves to death. The neoplastic natural killer (NK) cells usually harbor the Epstein-Barr virus (EBV) with a latent viral infection pattern type II; they often have a cytoplasmic CD3ε+ and surface CD3−, CD2+, and CD56+ immunophenotype, and they show complex genetic abnormalities affecting multiple tumor suppressor genes and oncogenes. We present a rare case of CD56-negative ANKL and review the clinical and laboratorial criteria for the diagnosis, as well as the available therapies. Case Presentation. A European 36-year-old male presented with acute onset fever, pallor, weakness, and jaundice. He had hepatosplenomegaly, severe pancytopenia, hepatic cytolysis, and very high serum lactic dehydrogenase levels. The bone marrow studies resulted in the diagnosis of an EBV-positive, CD56-negative ANKL. The patient failed to respond to gemcitabine and cisplatin-based polychemotherapy, dying three months later with leukemic meningitis and multiple cranial nerves palsies. Conclusions. The diagnosis of ANKL is difficult and requires both clinical suspicion and an extensive laboratorial approach. Absence of CD56 expression on the neoplastic NK cells may impose difficulties in the diagnosis, which requires morphological, immunophenotypic, histopathological, immunohistochemical, cytogenetic, and molecular studies. M. Guerreiro, F. Príncipe, M. J. Teles, S. Fonseca, A. H. Santos, E. Fonseca, P. Gomes, C. Marques, and M. Lima Copyright © 2017 M. Guerreiro et al. All rights reserved. Acquired Elliptocytosis as a Manifestation of Myelodysplastic Syndrome with Ring Sideroblasts and Multilineage Dysplasia Wed, 11 Oct 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/3625946/ Acquired elliptocytosis is a known but rarely described abnormality in the myelodysplastic syndromes (MDS). Here we report the case of an elderly male who was admitted to the hospital with chest pain, dyspnea, and fatigue and was found to be anemic with an elliptocytosis that had only recently been noted on peripheral smears of his blood. After bone marrow biopsy he was diagnosed with MDS with ring sideroblasts and multilineage dysplasia and acquired elliptocytosis. Here we report a rare case of acquired elliptocytosis cooccurring with MDS with ring sideroblasts and multilineage dysplasia. Jacob D. Kjelland, Denis M. Dwyre, and Brian A. Jonas Copyright © 2017 Jacob D. Kjelland et al. All rights reserved. Splenic Infarct and Pulmonary Embolism as a Rare Manifestation of Cytomegalovirus Infection Wed, 11 Oct 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/1850821/ Cytomegalovirus (CMV) is a type of herpes infection that has a characteristic feature of maintaining lifelong latency within the host cell. CMV manifestations can cover a broad spectrum from fever to as severe as pancytopenia, hepatitis, retinitis, meningoencephalitis, Guillain-Barre syndrome, pneumonia, and thrombosis. Multiple case reports of thrombosis associated with CMV have been reported. Deep vein thrombosis or pulmonary embolism is more common in immunocompetent patients while splenic infarct is more common in immunocompromised patients. However, here we report a female patient on low-dose methotrexate for rheumatoid arthritis who presented with both pulmonary embolism and splenic infarct. Prashanth Rawla, Anantha R. Vellipuram, Sathyajit S. Bandaru, and Jeffrey Pradeep Raj Copyright © 2017 Prashanth Rawla et al. All rights reserved. PAX5-Negative Classical Hodgkin Lymphoma: A Case Report of a Rare Entity and Review of the Literature Wed, 04 Oct 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/7531729/ Classical Hodgkin lymphoma (CHL) is recognized as a B-cell neoplasm arising from germinal center or postgerminal center B-cells. The hallmark of CHL is the presence of CD30 (+) Hodgkin and Reed-Sternberg (HRS) cells with dim expression of PAX5. Nearly all of the HRS cells are positive for PAX5. However, a small minority of HRS cells may lack PAX5 expression, which can cause a diagnostic dilemma. Herein we describe two cases of PAX5-negative CHL and review of the English literature on this very rare entity. It is crucial to be aware of this phenomenon, which in some cases may lead to misdiagnosis and may ultimately adversely affect patient’s management. Elham Vali Betts, Denis M. Dwyre, Huan-You Wang, and Hooman H. Rashidi Copyright © 2017 Elham Vali Betts et al. All rights reserved. Heterozygous Hemoglobin Sherwood Forest Causing Polycythemia Thu, 28 Sep 2017 13:42:14 +0000 http://www.hindawi.com/journals/crihem/2017/8174207/ Hemoglobin (Hb) Sherwood Forest is a rare high-affinity hemoglobin first described in 1977, arising from an Arg to Thr substitution at codon 104 of the beta chain. This hemoglobin variant has been identified in few individuals and has been associated with a compensatory erythrocytosis in the homozygous state. Prior scarce case reports have noted that heterozygotes for this variant are phenotypically normal. Here we present a patient who was evaluated in our hematology clinic for chronic erythrocytosis and was found to be heterozygous for Hb Sherwood Forest. No other primary or secondary cause of his polycythemia was identified. This is the first described case of heterozygous Hemoglobin Sherwood Forest causing erythrocytosis. Vikram M. Raghunathan, James N. Butera, and Diana O. Treaba Copyright © 2017 Vikram M. Raghunathan et al. All rights reserved. Polycythaemia Secondary to Hormone Replacement Therapy with Tibolone Wed, 27 Sep 2017 09:37:44 +0000 http://www.hindawi.com/journals/crihem/2017/3476349/ We present the case report of a patient with severe polycythaemia associated with tibolone. In our 65-year-old postmenopausal patient who initially presented with haemoglobin 203 g/L [115–160] and haematocrit 0.63 [0.32–0.47], the cessation of tibolone, a synthetic hormone replacement therapy, led to a dramatic and sustained resolution of this patient’s polycythaemia to normal haematological values. Tibolone possesses oestrogenic, androgenic, and progestogenic properties. Tibolone therapy may be an infrequently recognized contributor towards polycythaemia in postmenopausal patients presenting to haematology clinics. Laura Staples, Tamara Milder, and Philip Young-Ill Choi Copyright © 2017 Laura Staples et al. All rights reserved. Primary Cutaneous Follicle Centre Lymphoma with Hodgkin and Reed-Sternberg Like Cells: A Case Report and Review of the Literature Tue, 26 Sep 2017 09:54:37 +0000 http://www.hindawi.com/journals/crihem/2017/9549428/ An elderly woman with a complex medical history presented with a left forearm mass that slowly developed for several months. The excisional biopsy of this skin mass was remarkable for involvement by a follicle centre cell derived lymphoma with a nodular and diffuse pattern associated with a subset of scattered Hodgkin and Reed-Sternberg like cells. Fluorescence in situ hybridization studies did not detect the presence of IgH-bcl2 fusion transcript, and molecular studies were negative for immunoglobulin heavy chain gene rearrangements and EBV DNA sequences. Hodgkin and Reed-Sternberg like cells are rarely reported in FLs, and the association with primary cutaneous follicle centre lymphoma is extremely rarely seen. To our knowledge, our case is the second case of primary cutaneous follicle centre lymphoma with Hodgkin and Reed-Sternberg like cells. Fatima A. Aldarweesh and Diana O. Treaba Copyright © 2017 Fatima A. Aldarweesh and Diana O. Treaba. All rights reserved. A Case of Rituximab-Induced Necrotizing Fasciitis and a Review of the Literature Tue, 26 Sep 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/6971027/ Necrotizing fasciitis is a fulminant soft tissue infection characterized by rapid progression and high mortality. Rituximab is a generally well-tolerated immunosuppresive medication used for B-cell malignancies and some rheumatological disorders. We report a case of a 69-year-old male with chronic lymphocytic leukemia who suffered necrotizing fasciitis of his left lower extremity secondary to Clostridium septicum 7 weeks after treatment with rituximab. Despite immediate intravenous antimicrobial therapy and emergent fasciotomy with extensive debridement, his hospital course was complicated by septic shock and he required an above-the-knee amputation. Physicians need to be aware of the possibility of necrotizing fasciitis in patients presenting with skin infections after rituximab therapy. Abdullateef Abdulkareem, Ryan S. D’Souza, Oluwaseun Shogbesan, and Anthony Donato Copyright © 2017 Abdullateef Abdulkareem et al. All rights reserved. Upper Limb Deep Vein Thrombosis in Patient with Hemophilia A and Heterozygosity for Prothrombin G20210A: A Case Report and Review of the Literature Mon, 25 Sep 2017 10:02:09 +0000 http://www.hindawi.com/journals/crihem/2017/7290945/ Deep vein thrombosis (DVT) is a rare disease in patients with hemophilia A. We report a case of 22-year-old male with severe hemophilia A who presented to the emergency room with 5-day history of right arm pain that was attributed initially to bleeding event. In the absence of external signs of bleeding or hematoma and normal hemoglobin level, we suspected an underlying DVT. Doppler ultrasonography of the right upper limb revealed thrombosis of the subclavian vein and this was confirmed by CT venography. The d-dimer level was normal and investigations for prothrombotic state revealed heterozygosity for prothrombin G20210A mutation. Treatment with factor VIII and low molecular weight heparin led to successful resolution and marked improvement of his clinical condition. Fares Darawshy, Yosef Kalish, Issam Hendi, Ayman Abu Rmelieh, and Tawfik Khoury Copyright © 2017 Fares Darawshy et al. All rights reserved. Unusual Presentation of a Small-Cell Variant of Anaplastic Large-Cell Lymphoma Case: When a Septic Picture Is Not Sepsis Sun, 24 Sep 2017 09:57:54 +0000 http://www.hindawi.com/journals/crihem/2017/7292148/ We report a case of a small-cell variant of anaplastic large-cell lymphoma, with an unusual clinical presentation mimicking sepsis and a fulminant clinic course, in a 48-year-old Caucasian female. In this report, we discuss the diagnostic challenge, histopathologic features, and unique cytogenetic features of this case, in order to raise awareness of this rare presentation and emphasize the importance of meticulous peripheral smear examination and early bone marrow evaluation. Zhou Yu, Yifan Pang, Linda Wang, Daniel E. Ezekwudo, Foluso Ogunleye, Susanna S. Gaikazian, Mark Micale, James Huang, Ann Marie Blenc, and Ishmael Jaiyesimi Copyright © 2017 Zhou Yu et al. All rights reserved. γδ T-Cell Acute Lymphoblastic Leukemia/Lymphoma: Discussion of Two Pediatric Cases and Its Distinction from Other Mature γδ T-Cell Malignancies Sun, 24 Sep 2017 09:08:39 +0000 http://www.hindawi.com/journals/crihem/2017/5873015/ Gamma delta () T-cell antigen receptor (TCR) expression and its related T-cell differentiation are not commonly reported in T-cell acute lymphoblastic leukemia/lymphoma (T-ALL). Here we report two pediatric T-ALL cases and present their clinical features, histology, immunophenotypes, cytogenetics, and molecular diagnostic findings. The first patient is a two-year-old girl with leukocytosis, circulating lymphoblasts, and a cryptic insertion of a short-arm segment at 10p12 into the long-arm segment of 11q23 resulting in an MLL and AF10 fusion transcript, which may be the first reported in T-ALL. She responded to the chemotherapy protocol poorly and had persistent diseases. Following an allogeneic bone marrow transplant, she went into remission. The second patient is an eleven-year-old boy with a normal white cell count, circulating blasts, and a normal karyotype, but without any immature cellular markers by flow cytometric analysis. He responded to the chemotherapy well and achieved a complete remission. These cases demonstrate the diverse phenotypic, cytogenetic, and molecular aspects of T-ALL. Early T-precursor- (ETP-) ALL and their differential diagnosis from other mature T-cell leukemia/lymphomas are also discussed. Eric X. Wei, Vasiliki Leventaki, John K. Choi, Susana C. Raimondi, Elizabeth M. Azzato, Sheila A. Shurtleff, Menchu G. Ong, Diana M. Veillon, James D. Cotelingam, and Rodney E. Shackelford Copyright © 2017 Eric X. Wei et al. All rights reserved. Paroxysmal Nocturnal Hemoglobinuria in Pregnancy: A Dilemma in Treatment and Thromboprophylaxis Sun, 24 Sep 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/7289126/ Paroxysmal nocturnal hemoglobinuria (PNH) is a hematologic disorder characterized by an acquired somatic mutation in the phosphatidylinositol glycan class A gene which leads to a higher risk for increased venous and arterial thrombosis. Current treatment for PNH includes eculizumab. Pregnant patients who have PNH have higher risk for thrombosis and hemorrhage with both pregnancy and their underlying PNH. Treatment frequently poses conundrum. The safety and efficacy of eculizumab during pregnancy and breast feeding have not been extensively studied and contraception has been recommended due to potential for teratogenicity. We present a case of a patient who was safely on both eculizumab and modest prophylactic anticoagulation for 6 weeks post-partum. Arpan Patel, Athira Unnikrishnan, Martina Murphy, Robert Egerman, Sarah Wheeler, Ashley Richards, and John Wingard Copyright © 2017 Arpan Patel et al. All rights reserved. Plasmablastic Lymphoma: Case Report of Prolonged Survival of an Advanced Human Immunodeficiency Patient and Literature Review Sun, 24 Sep 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/9561013/ Clinical Practice Points. Plasmablastic lymphoma (PBL) is a rare and highly aggressive variant of diffuse large B cell lymphoma with median survival of advanced stage patients varying between 6 and 15 months in previous reports. We report here a human immunodeficiency virus-infected patient surviving over 12 years following treatment for advanced PBL with EPOCH chemotherapy and intrathecal therapy. This case highlights the potential for improved survival in PBL with intensive chemotherapy. Further, literature review suggests promising prospects utilizing novel targeted therapies to increase the rate of prolonged responses. Hind Rafei, Ehab El-Bahesh, Antoine Finianos, Min-Ling L. Liu, and Geraldine P. Schechter Copyright © 2017 Hind Rafei et al. All rights reserved. An Unsuspected Finding of t(9;22): A Rare Case of Philadelphia Chromosome-Positive B-Lymphoblastic Lymphoma Mon, 18 Sep 2017 00:00:00 +0000 http://www.hindawi.com/journals/crihem/2017/2413587/ While rare, cases of isolated extramedullary disease of B-cell Lymphoblastic Lymphoma (B-LBL) without morphologic bone marrow involvement have been described. In this report, we illustrate the case of an elderly gentleman who presented with isolated testicular and vertebral LBL involvement but had no morphologic bone marrow involvement. The initial plan of treatment was to treat along the lines of Philadelphia negative B-ALL/LBL. During this time, fluorescence in situ hybridization (FISH) and PCR testing for BCR-ABL1 rearrangements were being performed on the marrow specimens as a part of routine diagnostic workup. While the FISH returned negative, PCR testing unexpectedly detected BCR-ABL1 fusion transcripts at a low level of 0.48%. Given their presence, we performed FISH for BCR/ABL1 rearrangement in both testicular and L5 vertebral specimens which were 80–90% positive. He subsequently received rituximab, hyper-CVAD, and dasatinib, along with prophylactic intrathecal prophylactic chemotherapy. The patient achieved a prolonged remission but eventually relapsed, 4 years later. Had it not been for this fortuitous discovery, the patient would not have been treated with tyrosine kinase inhibitors. We emphasize that FISH and PCR testing for BCR-ABL1 rearrangement are integral to arriving at an accurate diagnosis and should be routinely tested on B-LBL biopsy specimens. Prajwal Boddu, C. Cameron Yin, Rashmi Kanagal-Shamanna, Guillin Tang, Beenu Thakral, Tapan Kadia, Marina Konopleva, Elias Jabbour, and Nitin Jain Copyright © 2017 Prajwal Boddu et al. All rights reserved. HbS-Sicilian (δβ)0-Thalassemia: A Rare Variant of Sickle Cell Sun, 17 Sep 2017 08:41:13 +0000 http://www.hindawi.com/journals/crihem/2017/9265396/ Sickle cell disease (SCD) is caused by a mutation in the sixth codon of the -globin gene on chromosome 11, which leads to a single amino acid substitution (glutamine to valine). Sickle-(δβ)0-thalassemia is a rare variant of sickle cell disease (delta-beta thalassemia occurring in association with sickle hemoglobin, HbS), sparsely reported in literature, and has been associated with symptomatology necessitating careful monitoring and follow-up. We describe a patient who presented with a newborn screen reported as “FS” and a negative family history for sickle cell disease and sickle cell trait. Subsequent gene sequencing studies demonstrated the presence of Sickle-(δβ)0-thalassemia. Clinical course has remained relatively stable for this patient now at 18 months of age without any SCD related symptomatology or complications. As this is a rare variant of SCD with potential complications, it is important to establish diagnosis towards planning comprehensive care. Grace Onimoe and Genine Smarzo Copyright © 2017 Grace Onimoe and Genine Smarzo. All rights reserved.