Hematology Reports

3 pages, 186 KiB

Open AccessCase Report

A 80-Year-Old Woman with B-Cell Prolymphocytic Leukemia

by Alparslan Merdin, Jale Yildiz, Mehmet Sinan Dal, Merih Kızıl Çakar, Hikmetullah Batgi, Emre Tekgündüz, Aykut Onursever and Fevzi Altuntaş

Hematol. Rep. 2017, 9(1), 6995; https://doi.org/10.4081/hr.2017.6995 - 22 Mar 2017

Cited by 1 | Viewed by 355

Abstract

Prolymhocytic leukemia (PLL) is a rare subtype of lymphocytic leukemias and its cells are immature lymphocytes. It is divided into 2 subgroups: T-PLL and B-PLL according to the lymphocytic origin of the cells. Discriminating B-PLL from other diseases with clinically-similar features is important [...] Read more.

Prolymhocytic leukemia (PLL) is a rare subtype of lymphocytic leukemias and its cells are immature lymphocytes. It is divided into 2 subgroups: T-PLL and B-PLL according to the lymphocytic origin of the cells. Discriminating B-PLL from other diseases with clinically-similar features is important because of the different treatment approaches and follow-up programs. Hereby, we report a 80-year-old woman presenting with fatigue, leucocytosis and mild anemia. Her peripheral blood smear evaluation revealed 85% prolymphocytes with moderately condensed nuclear chromatin, prominent nucleoli, and a faintly basophilic cytoplasm. Positron emission tomography-computed tomography showed mediastinal lymph nodes with cervical lymph nodes. There was no pathological FDG involvement in the spleen. Bone marrow aspiration smear exhibit atypical wide lymphocytes with prominent nucleoli and abundant agranular cytoplasm. Flow cytometry analysis revealed positive CD5⁺, CD19⁺, CD20⁺, CD22⁺, CD11c⁺, CD25⁺, CD79a⁺ and CD79b⁺. Fluorescence in situ hybridization technique analysis reveals no t(11;14). Bone marrow biopsy revealed interstitially distributed atypical cells with wide nucleus and prominent nucleolus. Full article

4 pages, 707 KiB

Open AccessCase Report

An Unusual Case of Primary Extranodal Lymphoma of the Gallbladder

by Roberto Pezzuto, Davide Di Mauro, Luca Bonomo, Amita Patel, Edoardo Ricciardi, Andrea Attanasio and Antonio Manzelli

Hematol. Rep. 2017, 9(1), 6972; https://doi.org/10.4081/hr.2017.6972 - 22 Mar 2017

Cited by 5 | Viewed by 381

Abstract

Primary gallbladder lymphoma is an extremely rare disease. We report a case of a 63 year-old woman who has been admitted with gradual onset abdominal pain in the upper right quadrant and in the suprapubic region, nausea and malaise. According to the computed [...] Read more.

Primary gallbladder lymphoma is an extremely rare disease. We report a case of a 63 year-old woman who has been admitted with gradual onset abdominal pain in the upper right quadrant and in the suprapubic region, nausea and malaise. According to the computed tomography scan of the abdomen, which was suggestive of chronic cholecystitis, she was treated conservatively. A laparoscopic cholecystectomy was performed 5 months later and the histological examingation of the gallbladder showed a low grade small lymphocytic lymphoma. The patient has been taken over by the hematology team who kept her under surveillance as no further treatment was deemed as necessary. The purpose of this paper is to report a rare case of primary gallbladder lymphoma and to demonstrate that a laparoscopic cholecystectomy may be a valid treatment for this disease. Full article

3 pages, 551 KiB

Open AccessCase Report

Paroxysmal Nocturnal Hemoglobinuria in the Differential Diagnosis of Thrombocytopenia

by Fusun Gediz, Bahriye Kadriye Payzin, Ozlem Zekiye Cakmak, Yusuf Uzum, Damla Ernur and Fahri Sahin

Hematol. Rep. 2017, 9(1), 6862; https://doi.org/10.4081/hr.2017.6862 - 22 Mar 2017

Viewed by 463

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a disease which diagnosis may be delayed due to variable clinical findings. We describe herein a case of PNH in a 21 year old woman who admitted with complaints of chronic weakness, intermittent spontaneous ecchymoses, and an intermittent [...] Read more.

Paroxysmal nocturnal hemoglobinuria (PNH) is a disease which diagnosis may be delayed due to variable clinical findings. We describe herein a case of PNH in a 21 year old woman who admitted with complaints of chronic weakness, intermittent spontaneous ecchymoses, and an intermittent abdominal pain. On laboratory tests thrombocytopenia and iron deficiency anemia without any clinical findings were found. Flow cytometric evaluations showed a PNH clone of 15% for erythrocytes, 64% for monocytes, and 60% for granulocytes. The patient was diagnosed with PNH and an eculizumab therapy was initiated. Following initiation of eculizumab therapy, the frequency of abdominal pain attacks decreased, hemoglobin level normalized, and platelet values increased slightly. In patients submitting with a triad of symptoms such as thrombocytopenia, iron deficiency anemia, and abdominal pain attacks of unknown etiology we suggest considering PNH. We also encourage physicians to share their similar observations in order to raise the knowledge on infrequent presentations of PNH. Full article

4 pages, 803 KiB

Open AccessCase Report

A Fatal Case of Immune Hyperhemolysis with Bone Marrow Necrosis in a Patient with Sickle Cell Disease

by Matthew S. Karafin, Arun Singavi, Susan T. Johnson and Joshua J. Field

Hematol. Rep. 2017, 9(1), 8-11; https://doi.org/10.4081/hr.2017.6934 - 01 Mar 2017

Cited by 7 | Viewed by 325

Abstract

In patients with sickle cell disease, hyperhemolysis is a rare but life-threatening complication of transfusion. In this case report, we describe a 61 year-old woman with hemoglobin sickle cell (SC) disease and history of alloimmunization who developed hyperhemolysis associated with a transfusion. She [...] Read more.

In patients with sickle cell disease, hyperhemolysis is a rare but life-threatening complication of transfusion. In this case report, we describe a 61 year-old woman with hemoglobin sickle cell (SC) disease and history of alloimmunization who developed hyperhemolysis associated with a transfusion. She was found to have a warm and a clinically-significant cold autoantibody. Severe anemia (Hb 2.7 g/dL) with reticulocytopenia and thrombocytopenia prompted a bone marrow biopsy, which demonstrated extensive bone marrow necrosis. Despite treatment, the bone marrow failure did not improve and the patient died on hospital day 38. This case illustrates the potential risks of transfusion in a patient with sickle cell disease, especially one with previous hemolytic reactions. While uncommon, hyperhemolysis can cause death, in this case by extensive bone marrow necrosis. In patients with sickle cell disease, judicious use of red cell transfusions with phenotypically-matched units can diminish, but never completely abrogate, the risks associated with transfusion. Full article

4 pages, 907 KiB

Open AccessCase Report

Hemophagocytic Lymphohistiocytosis, an Unclear Nosologic Entity: Case Report of an Adult Man with Rising of Amylase and Lipase and Spinal Cord Infiltration

by Moris Sangineto, Antonio Perrone, Pasquale Agosti, Viera Boccuti, Anna Campobasso and Carlo Sabbà

Hematol. Rep. 2017, 9(1), 4-7; https://doi.org/10.4081/hr.2017.6859 - 01 Mar 2017

Cited by 2 | Viewed by 300

Abstract

Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, [...] Read more.

Here we present the case of a 57-years old patient affected by hemophagocytic lymphohistiocytosis (HLH), a rare disease characterized by an uncontrolled immune activation, resulting in clinical and biochemical manifestations of extreme inflammation. In a previous hospitalization, the patient showed fever, hepato-splenomegaly, pancytopenia, hyperferrtitinemia, lymphadenopathy and cholestasis. No diagnosis was done, however, he totally recovered after splenectomy. Eight months later, he relapsed, showing also hypofibrinogenemia, hypertriglyceridemia, hemophagocytic signs in bone marrow, cholestatic jaundice, high LDH and high PT-INR. Interestingly, he presented increased levels of amylase and lipase in absence of radiologic signs of pancreatitis. He was treated with Dexamethasone and Cyclosporine according to HLH-2004 guidelines. The clinical and biochemical manifestations disappeared in a few weeks, but he was newly hospitalized for lower limbs hypotonia caused by a hemophagocytic lesion of the cauda equina and lumbar cord. The death occurred in a few days, despite the immunosuppressive treatment. Full article

3 pages, 669 KiB

Open AccessCase Report

Hemoglobin Willamette (b51 Pro → Arg): Case Report and Literature Review

by Orivaldo Alves Barbosa, Matheus Martins de Sousa Dias, Saymon Medeiros Távora, Gentil Claudino de Galiza Neto, Jacqueline Holanda de Souza and Herivaldo Ferreira da Silva

Hematol. Rep. 2017, 9(1), 6953; https://doi.org/10.4081/hr.2017.6953 - 01 Mar 2017

Cited by 1 | Viewed by 326

Abstract

We report a case of hemoglobin (Hb) Willamette (β51 Pro → Arg) in the Hematology Department of a tertiary hospital in Fortaleza, Northeast of Brazil. A literature review of the cases described in health sciences databases using as a descriptor Hb Willamette was [...] Read more.

We report a case of hemoglobin (Hb) Willamette (β51 Pro → Arg) in the Hematology Department of a tertiary hospital in Fortaleza, Northeast of Brazil. A literature review of the cases described in health sciences databases using as a descriptor Hb Willamette was performed, revealing 12 reported cases, of which only one presented with anemia. Herein, we describe a case of a female 29 years old, with hemoglobinopathy Willamette presenting clinically with anemia, having the lowest hemoglobin rate of the published cases. The relatives of the patient were evaluated andthe patient’s mother corresponded to the first description of the association between Hb Willamette and HbC. Among the hemoglobinopathies, hemoglobin Willamette is an extremely rare disease; therefore it is important to analyze its clinical and laboratory manifestations for accurate diagnosis and assessment of potential interactions with other genetic variants. Full article

4 pages, 605 KiB

Open AccessCase Report

Successful Intrathecal Chemotherapy Combined with Radiotherapy Followed by Pomalidomide and Low-Dose Dexamethasone Maintenance Therapy for a Primary Plasma Cell Leukemia Patient

by Yusuke Yamashita, Shinobu Tamura, Takehiro Oiwa, Hiroshi Kobata, Kodai Kuriyama, Toshiki Mushino, Shogo Murata, Hiroki Hosoi, Akinori Nishikawa, Nobuyoshi Hanaoka and Takashi Sonoki

Hematol. Rep. 2017, 9(1), 28-31; https://doi.org/10.4081/hr.2017.6986 - 23 Feb 2017

Cited by 5 | Viewed by 349

Abstract

Primary plasma cell leukemia (PPCL) is a rare aggressive variant of plasma cell disorder and frequently presents with extramedullary disease. Central nervous system (CNS) involvement with PPCL has an extremely poor prognosis. We describe a 46-year-old man with PPCL treated with a combination [...] Read more.

Primary plasma cell leukemia (PPCL) is a rare aggressive variant of plasma cell disorder and frequently presents with extramedullary disease. Central nervous system (CNS) involvement with PPCL has an extremely poor prognosis. We describe a 46-year-old man with PPCL treated with a combination of lenalidomide, bortezomib, and dexamethasone as induction therapy following upfront allogeneic stem cell transplantation (allo-SCT). Despite achieving a very good partial response, the patient suffered from an isolated CNS relapse 12 months after allo-SCT. He was immediately started on concurrent intrathecal chemotherapy (IT) and cranial irradiation (RT). Subsequently, pomalidomide and low-dose dexamethasone (Pd) were given as maintenance therapy. He has been without CNS recurrence for more than 18 months. Our case suggests that concurrent IT and RT followed by Pd maintenance therapy may be an effective option to control CNS relapse of PPCL after allo-SCT. Full article

6 pages, 607 KiB

Open AccessArticle

The Efficacy of Colchicine and Dapsone Combination Therapy in Relapsed Immune Thrombocytopenia

by Thanawat Rattanathammethee, Wasan Theerajangkhaphichai, Ekarat Rattarittamrong, Sasinee Hantrakool, Chatree Chai-Adisaksopha, Lalita Norasetthada and Adisak Tantiworawit

Hematol. Rep. 2017, 9(1), 22-27; https://doi.org/10.4081/hr.2017.7034 - 23 Feb 2017

Cited by 3 | Viewed by 489

Abstract

The aim of the present paper is to evaluate the efficacy and safety of colchicine and dapsone combination therapy in cases of steroid-dependent, relapsed and refractory immune thrombocytopenia (ITP). This is a retrospective study of ITP patients who attended the Hematology Clinic at [...] Read more.

The aim of the present paper is to evaluate the efficacy and safety of colchicine and dapsone combination therapy in cases of steroid-dependent, relapsed and refractory immune thrombocytopenia (ITP). This is a retrospective study of ITP patients who attended the Hematology Clinic at Chiang Mai University Hospital (Thailand) from 1 January 2008 to 30 September 2014. Medical records and clinical data were reviewed for efficacy and adverse effects. Sixty-four ITP patients received the combination therapy. The median age was 46 years and 70.3% were female. The majority (65.6%) were relapsed ITP patients. Median platelet count before starting treatment was 22.6 × 109/L. The response rate was 82.8%, with 75.0% of patients having a complete response. Median time to response was 8 weeks. The response rate was higher in relapsed patients (90.4%) compared to refractory (61.5%) and steroid-dependent patients (77.8%). Steroid treatment was discontinued in 30 patients (50%) following combination therapy. The most common side effect was hemolysis due to dapsone which was found in eight patients (12.5%). We can therefore conclude that combination therapy with colchicine and dapsone is an alternative second-line therapy option in relapsed ITP cases with acceptable side effects. Full article

3 pages, 550 KiB

Open AccessArticle

Can Ratio of Neutrophil-Tolymphocyte Count and Erythrocyte Sedimentation Rate in Diabetic Foot Infection Predict Osteomyelitis and/or Amputation?

by Oktay Yapıcı, Hande Berk, Nefise Öztoprak, Derya Seyman, Alper Tahmaz and Alparslan Merdin

Hematol. Rep. 2017, 9(1), 19-21; https://doi.org/10.4081/hr.2017.6981 - 23 Feb 2017

Cited by 23 | Viewed by 508

Abstract

The aim of this study was to search for any relations between the neutrophil-tolymphocyte ratio (NLR) and the development of osteomyelitis and the need for amputation in patients with diabetic foot infection (DFI). All data included DFI patients who were hospitalized in our [...] Read more.

The aim of this study was to search for any relations between the neutrophil-tolymphocyte ratio (NLR) and the development of osteomyelitis and the need for amputation in patients with diabetic foot infection (DFI). All data included DFI patients who were hospitalized in our Infectious Diseases Clinic between 2012 and 2015 and who were classified according to International Classification Disease Code System. 75 patients were analyzed in the study. The DFI patients were stratified into 3 groups of whom had amputation procedure, whom had only debridement/drainage procedure and whom had any surgery procedure. Sidac post hoc analysis was used to perform the effects of NLR, C-reactive protein, erythrocyte sedimentation rate and glycosylated hemoglobin on the surgery procedure status. The DFI patients were also stratified into two another separate group for another analysis to search for the effect of NLR values on the development of osteomyelitis. The mean value of NLR in the amputated patients’ group (15.7 ± 10.3 was significantly higher than those with debridement procedure (9.9 ± 5.6) and those without any surgery (6.0 ± 2.8) (p = 0.001). NLR values were also found significantly higher in patients with osteomyelitis in the second analysis (p = 0.004). In this study, the NLR was found to have a predictive value on the development of osteomyelitis and on the progression to amputation in patients with DFI. Full article

3 pages, 550 KiB

Open AccessBrief Report

Impact of CRAB Symptoms in Survival of Patients with Symptomatic Myeloma in Novel Agent Era

by Aya Nakaya, Shinya Fujita, Atsushi Satake, Takahisa Nakanishi, Yoshiko Azuma, Yukie Tsubokura, Masaaki Hotta, Hideaki Yoshimura, Kazuyoshi Ishii, Tomoki Ito and Shosaku Nomura

Hematol. Rep. 2017, 9(1), 16-18; https://doi.org/10.4081/hr.2017.6887 - 23 Feb 2017

Cited by 21 | Viewed by 978

Abstract

The acronym CRAB summarizes the most typical clinical manifestations of multiple myeloma, these being hypercalcemia, renal failure, anemia, and bone disease. CRAB can be used to distinguish between active, symptomatic multiple myeloma and monoclonal gammopathy of undermined significance or smoldering myeloma. The distinction [...] Read more.

The acronym CRAB summarizes the most typical clinical manifestations of multiple myeloma, these being hypercalcemia, renal failure, anemia, and bone disease. CRAB can be used to distinguish between active, symptomatic multiple myeloma and monoclonal gammopathy of undermined significance or smoldering myeloma. The distinction is relevant not only for classification and diagnosis but also for therapy. CRAB factors influence the prognosis of multiple myeloma. However, it is unclear whether the presence of CRAB factors has an influence on the prognosis of myeloma treated with novel agents. In the current study, patients with hypercalcemia and bone disease showed a significantly worse prognosis, whereas anemia and renal failure showed no difference in survival. Novel agents used for treatment of patients with renal failure suggested a favorable outcome compared with conventional therapy. Bone disease was the most common factor and may have the strongest prognostic value in symptomatic myeloma patients using novel agents. Full article

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Hematol. Rep., Volume 9, Issue 1 (February 2017) – 10 articles

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