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Παρασκευή, 26 Ιουλίου 2019

Imatinib Induced Complete Remission of Psoriasis in a Patient with Chronic Myeloid Leukemia

Clinical Characteristics and Treatment Outcome of Hypocellular Acute Myeloid Leukemia Based on WHO Classification

Abstract

The hypocellular acute leukemia is very rare atypical leukemia with frequency of 5–7% among patients with acute leukemias. It mainly occurs in older patients and usually has a myeloid phenotype. It is still unclear whether the outcome of hypocellular acute myeloid leukemia is less favorable than adult acute myeloid leukemia with normal cellularity. We retrospectively analyzed all hypocellular acute myeloid leukemias which were treated in 16 years period, between January 1998 and December 2014. There were 33 patients, 21 male and 12 female. The median age of the patients was 58.9 years (ranging from 19 to 88 years) and median cellularity of bone marrow was 16%. All patients presented with cytopenias with median white blood cell count 1.9 × 109/l, platelets 47.2 × 109/l and hemoglobin 85.9 g/l. Nineteen patients were treated with standard 3 + 7 protocol (daunoblastin 45 mg/m2 1, 3, 5 days, cytosin-arabinozide 100 mg/m2/12 h for 7 days), 5 patients with HDAC protocol and, 3 (9%) with low dose cytosin-arabinoside and in 6 (18.1%) patients only supportive therapy was applied. One patient died on 34 day after treatment with HiDAC, 3 patients after treatment with 3 + 7 regimen in full doses on days 23, 35, and 58 days. Complete remission was achieved in 20/33 (60.60%) patients, with median duration of 14 months. Median overall survival (OS) of the entire cohort was 16 months, and for the treated group 21 months (range 5–67 months). Median OS of patients treated with low dose cytosine-arabinoside was 6 months. The advanced age (p = 0.009, KK = − 0.46, Log rank, p = 0.031) as well as therapy options (Log rank p < 0.0001) shows a significant correlation with OS. We report a cohort of patients with hypocellular acute myeloid leukemia who responded to standard induction chemotherapy as are in standard acute myeloid leukemia.

Synchronous T-Non Hodgkins Lymphoma and Multiple Myeloma: A Rare Association

Imatinib Induced Blue Nails

Response to Immunosuppressive Therapy in Acquired Aplastic Anaemia: Experience of a Tertiary Care Centre from Eastern India

Abstract

The current study was conducted to assess response to immunosuppressive therapy (IST) in acquired aplastic anaemia (AA). It was a retrospective and prospective observational study. Patients were diagnosed as per standard international guidelines and IST was started as per standard protocol. Patients were followed up at 3 months and 6 months for assessment of response as per published standard guidelines. Total 76 cases were included in the study. The median age of the study population was 36 years with a range of 6–66 years with a male to female ratio of 2.04:1. Most common clinical presentation was pallor followed by bleeding. Commonest type of disease in the study group was severe AA. Among total 76 patients, 32 patients received Atgam and 44 patients received Thymogam. Within 3 months of ATG administration, 4 patients died and 1 patient was lost to follow up. At 3 months, 2 (2.63%) patients were on complete response (CR), 32 (42.10%) patients were in partial response (PR) and 37 (48.68%) patients were on no response (NR). Overall response (OR) at 3 months was 44.73%. At 6 months 5 (6.57%) patients were in CR, 43 (56.57%) patients in PR and 23 (30.26%) patients in NR; the OR was 63.14%. Overall response at 3 months was 44.73% and overall response at 6 months was 63.14%. The study revealed better overall survival for patients with ATGAM treatment than THYMOGAM treatment arm.

Dysplastic Erythroblastic Islands in Paroxysmal Nocturnal Hemoglobinuria (PNH)

Dissecting Primary Erythrocytosis Among Polycythemia Patients Referred to an Indian Armed Forces Hospital

Abstract

Referrals for evaluation of polycythemia cases have increased since the hemoglobin (Hb) thresholds for diagnosis of Polycythemia Vera (PV) have been lowered by WHO. The current study enrolled patients of age > 18 years from the Indian Armed Forces or their family members with polycythemia from November 2016 to October 2018. After exclusion of secondary causes, 49 patients were diagnosed as Primary Erythrocytosis (PE). The patients were classified into two groups: PV and Idiopathic Erythrocytosis (IE) and a systematic comparison of clinical and laboratory features of the two groups was done. The prevalence of PV in PE was 20.4% (10 of 49) while the rest 39 (79.6%) had IE. Seven PV patients had JAK2V617F mutation, one had JAK2Exon12 mutation, and two were JAK2 negative PV. Nine of 10 (90%) PV patients had Hb > 18.5 g/dl, while only 21 of 39 (53.8%) IE patients had Hb > 18.5 g/dl (p = 0.06). None of the JAK2 mutated patients had Hb < 18.5 g/dl. We conclude that PV is more prevalent in patients of PE with Hb > 18.5 g/dl. Most patients with Hb between 16.5–18.5 g/dl would still be classified as IE. We advocate the need for further studies evaluating the utility of investigating all patients of PE with the revised WHO Hb threshold as well as studies on genetic profile of IE patients from India.

Phenotypic Diversity and Clinico-Hematological Profile of Hb E-Beta Thalassemic Children

Abstract

Hb E-Beta thalassemia is a disease with marked clinical diversity. In this study, phenotypic diversity of Hb E-β thalassemia children were analysed by studying the clinical and hematological parameters. This was a cross sectional study done in one and a half year period in the department of Pediatrics of a tertiary care teaching hospital. Participants were 62 Hb E-β thalassemic children of age group 1 month to 18 years coming to the Thalassemia day care centre for blood transfusion. Data collected from history, examination findings and investigation reports were analyzed. M:F ratio was 1.07:1; 71% children were above 5 years of age. 90.3% children were Hindu. In 66.1% children, Hb level was below 5 gm/dl at the time of diagnosis. Mean HbF level was 32.6% ± 11.2. Stunting was seen in 64.5%. Average liver and spleen size were 2.5 and 4.4 cm respectively. Beside pallor, most common clinical findings were splenomegaly (93.5%), facial deformity (87%), dusky skin color (82.5%) and hepatomegaly (75.8%). 1.6% children were mild, 43.5% children were moderate and 54.8% children were of severe type. In our study there was no significant correlation between severity of the disease and HbF level (r = 0.0853, p = 0.0509). Age at the time of diagnosis, hemoglobin level at the time of first transfusion, age at receiving first blood transfusion, requirement of blood transfusion, spleen size and growth, are some factors affecting severity of the disease. But severity cannot be assessed by considering only one clinical or hematological parameter but by considering several parameters together.

Auer Rod-Like Inclusions in Light Chain Myeloma: A Rare Morphological Feature

T-Cell Acute Lymphoblastic Leukemia with Massive Vitreo-Retinal Infiltration and Neovascular Glaucoma

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