Myelodysplastic syndromes are a heterogeneous group of clonal disorders characterized by peripheral blood cytopenia and normal or hypercellular bone marrow with dysplasia in more than one blood cell lineage, unfavorable prognosis, and lack of response to treatment. We present the case of a 12-year-old male patient who was referred to the Hematology and Oncology Department of Pediatric Clinic I Târgu- Mures in May 2016, with splenomegaly and pancytopenia. The osteomedullary biopsy revealed myelofibrosis, discrete dysplasia of the myeloid series and megakaryocytes, blasts CD34+ approximately 10%, which led to the diagnosis of myelodysplastic syndrome with myelofibrosis. The myeloid precursors indicated a high risk of transformation into acute myeloid leukemia, so chemotherapy associated with corticosteroids was started, leading to slight improvements. Although myelodysplastic syndrome associated with myelofibrosis is rare at this age, despite the treatment and favorable progression in the case presented, in the absence of hematopoietic stem cell transplantation the prognosis remains unfavorable.
Methotrexate (MTX) is a cytostatic agent used in oncology. Because of its immunosuppressive properties, MTX is also used in autoimmune disorders. Low-dose MTX regimens in the treatment of rheumatoid arthritis and severe psoriasis are considered to be safe. However, pharmacovigilance centers warn of serious and even fatal incidents due to errors in oral MTX administration. The aim of this case series presentation was to identify the specific factors related to the development of adverse drug reactions (ADRs) induced by MTX. A prospective pharmacovigilance study was conducted at the Clinic of Dermatology, University Hospital, Stara Zagora. We report 3 cases of patients with psoriasis vulgaris in which severe haematological abnormalities associated with previous administration of MTX were detected during hospitalization. A 73-year old female with malaise, vomiting and oral ulcers who had taken approximately 120 mg MTX was found to have pancytopenia. A 59-year old male hospitalized for psoriatic erythroderma who had erroneously taken 10 mg MTX daily instead of weekly for 8 days, was diagnosed with bicytopenia and toxic hepatitis. An 88-year old male with psoriatic arthritis presented with aphthous stomatitis, erosive crusted lesions, ecchymoses and aplastic anemia 2 weeks after treatment with 12.5 mg MTX once weekly plus i.m. Movalis®, followed by Diclophenac Duo®. The main predisposing factors for the development of these ADRs were patient-related dosage errors and concomitant administration of NSAIDs. Safe use of oral MTX requires clear dosing instructions and strict patient compliance. Potential drug interactions of MTX with other drugs should also be considered.
Introduction: Fanconi anemia is an autosomal recessive disease characterized by congenital abnormalities, defective haematopoiesis, and a high risk of developing acute myeloid leukaemia, myelodysplastic syndrome and cancers. FA was first described in 1927 by the Swiss pediatrician Guido Fanconi. The diagnosis is based on morphological abnormalities, hematologic abnormalities (pancytopenia, macrocytic anemia and progressive bone marrow failure) and genetic tests (cariograma).
Case report: We present the case of a child with Fanconi anemia. Although skin and bone morphological abnormalities were present from birth, diagnosis was suspected at 11 years old.
Conclusions: Fanconi anemia is a heterogeneous condition that can present a variety of congenital defects but invariably results in defective haemopoiesis, which is the major cause of morbidity and mortality.
pancytopenia and BM failure, caused by destruction of hematopoietic cells by the antigen-speciﬁc T lymphocytes. [ 2 ] Our previous studies have demonstrated that suppressor T lymphocytes (mainly CD8 + T cells) have a dramatic increase and hyperfunction in the majority of patients with AA. Those activated T lymphocytes have obvious inhibitory effect on the growth of BM cells in in vitro experiments. Meanwhile, the quantity of activated effector T cells (CD8 + HLA-DR + ) was elevated in patients with AA. [ 3 ] While after IST [antithymocyte globulin (ATG)+ cyclosporine (CSA
Orthomyxoviruses, Rhabdoviruses VI ssRNA-RT viruses (+ strand or sense) RNA with DNA intermediate in life-cycle Retroviruses VII dsDNA-RT viruses DNA with RNA intermediate in life-cycle Hepadnaviruses Role of viruses in hematology From the hematologist point of view, viruses can play a major role in four conditions: causing infections; causing lymphoproliferations and/ or malignancies; causing (pan)cytopenia; and used as vectors in treatment (e.g., gene therapy, CAR-T cells) ( Tab. II) Table II Role of viruses in hematology Role Examples Causing infections Episodic infections
Introduction Aplastic anemia (AA) is a bone marrow hematopoietic dysfunction disease caused by one or more factors, which is characterized by low bone marrow nucleated cell proliferation and peripheral blood pancytopenia, without abnormal cell infiltration or myelofibrosis.[ 1 ] The main clinical manifestations consist of different degrees of anemia, hemorrhage and infection.[ 2 ] Up to now, the Camitta standard is still used internationally. The severity of AA is defined according the blood count parameters and bone marrow findings, which can be divided into
was not performed due to the patient’s overall bad condition. CMV test by PCR was negative at that time. Immunosuppressive treatment consisting of methylprednisolone, mycophenolate mofetil, and tacrolimus was initiated; however, the disease persisted. A severe variant of steroid-resistant GVHD was diagnosed. As a consequence, second CMV reactivation was demonstrated (Day +110; copy number: 37,000/mL). Treatment with ganciclovir was initiated, but the patient showed deepening of her pancytopenia and became dependent on transfusion. Bone marrow examination revealed
hyclate treatment of experimental canine ehrlichiosis followed by challenge inoculation with two Ehrlichia canis strains. Antimicrob Agents Chemother 1998,42:362-368. 25. Hildebrandt PK, Huxsoll DL, Walker JS, Nims RM, Taylor R, Andrews M. Pathology of canine ehrlichiosis (tropical canine pancytopenia). Am J Vet Res 1973,34:1309-1320. 26. Harrus S, Waner T, Weiss DJ, Keysary A, Bark H. Kinetics of serum antiplatelet antibodies in experimental acute canine ehrlichiosis. Vet Immunol Immunopathol 1996,51:13-20. 27. Harrus S, Waner T, Keysary A, Aroch I, Voet H, Bark H
after, the patient started complaining of headache with photophobia and was admitted to a regional hospital a day later with high fever, confusion and pancytopenia. A head CT scan revealed diffuse hypodensities involving the right parieto-temporo-occipital region, spreading into the frontal lobe and across the corpus callosum into the left parieto-occipital lobe. A hypodensity in the central region of the cerebellum was present as well. There were no mid-line brain shifts or herniations and the ventricular system was normal. Progression of the lymphoma to the CNS was
Objective: To evaluate the clinical presentation, possible etiological factors, management and
outcome of patients in our hospital with extrahepatic portal vein obstruction (EHPVO). Materials
and Methods: This study included patients with EHPVO followed up in our department during
last 10 years. Patients of cirrhosis with EHPVO were excluded. Patients’ clinical presentation,
etiology of EHPVO, management and outcome results were analyzed. Results: Of 30 patients,
19 (67.9%) were males. Median age was 12 years. Of 14 patients who underwent liver biopsy
9 had histological activity index stage of 1/6. History of omphalitis and pulmonary tuberculosis
was present in one case each. Of 22 patients with the available thrombophilia profile, nine
patients had a deficiency of protein C, five patients had a deficiency of protein S, one each
had reduced level S of anti-thrombin III and factor V mutation. The predominant presenting
symptom was hematemesis (15 patients, 53.6%). Seven patients (25%) had splenomegaly.
Three patients (10.7%) had no esophageal varices on endoscopy. Three patients underwent
splenectomy due to severe pancytopenia. Endoscopic retrograde cholangipancreatography
was performed in four patients (14.3%) due to portal biliopathy. Common bile duct stenting was
performed in all four patients. Of them, one patient underwent splenorenal shunt operation for
indication of hemobilia. One patient died at the age of 40 years, due to cholangitis and sepsis.
Conclusions: Results from this study show that the anticoagulant deficiency is a common
cause of EHPVO in our setup. Hematemesis is a common presenting symptom. Some of these
patients have symptomatic portal biliopathy.