RESUMO
Pure erythroid leukemia (AML-M6) is a rare variant of acute myeloid leukemia (AML) with predominant erythroid lineage proliferation. The incidence of AIDS defining cancers including Kaposi sarcoma and non-Hodgkins lymphoma are on declining trends due to effective use of HAART (Highly Active Antiretroviral Therapy). Correspondingly, there have been increasing cases of leukemia in persons living with HIV. Our case is a 43 years old male living with HIV who was admitted with high grade fever and mucosal bleeds. On examination, he had periorbital swelling and ecchymosis with hepatosplenomegaly. The laboratory investigations revealed bicytopenia with high ferritin, low fibrinogen and hypertriglyceridemia. A diagnosis of hemophagocytic lymphohistiocytosis (HLH) with H score of 222 was made. Bone marrow examination revealed hypercellular marrow with more than 80% cells of erythroid lineage with 47% proerythroblasts. Suggesting pure erythroid leukemia (AML-M6). This diagnosis with secondary HLH carries a very poor prognosis in persons living with HIV.
RESUMO
Thrombotic complications in liver abscess are less commonly identified in the literature. In this prospective observational study, vascular thrombosis was detected in 21.9% cases. However, there was no significant difference in outcome with or without vascular thrombosis, properly treated.
Assuntos
Abscesso Hepático , Trombose , Humanos , Prevalência , Veia Porta , Trombose/diagnóstico , Trombose/epidemiologia , Trombose/etiologia , Abscesso Hepático/epidemiologia , Abscesso Hepático/complicações , Estudos ProspectivosRESUMO
We aimed to estimate the prevalence of anemia in children with nephrotic syndrome (NS), determine its etiology, and correlate severity with disease duration and response to steroids. This was a prospective cohort study carried from 15th July 2019-14th July 2021 at the pediatric nephrology clinic, of a teaching hospital in India. We screened children aged 3 months-18 years with NS for eligibility. We excluded those suffering from chronic kidney disease and, on haematinics. All children underwent investigations for evaluation of nephrotic syndrome and anemia. To define the clinical phenotype of nephrotic syndrome, the patients were classified as infrequent relapsers, frequent relapsers, steroid dependent and steroid resistant NS as per ISPN guidelines. Children were followed up at least for a period of one year to define their response to steroids. A total of 125 children were finally analysed for all treatment outcomes. Of 125, 37 (30%) children presented with the first episode of NS. Remaining 88 were follow up cases of NS. Of 125 children, 41 (33%) were found to be anemic as per the WHO criteria. Iron deficiency anemia was found in 21 (51%) children. Steroid resistance was twice more prevalent in the anemic group compared to the non-anemic group, 7.3% vs 4.8% respectively, however this difference was not statistically significant, p = 0.65. Anemic group had a trend of higher no. of children receiving antihypertensives compared to non-anemics (38 (93%) vs. 67 (80%), p = 0.07. CONCLUSION: Iron deficiency anemia was the commonest cause of anemia and, anemia and need for anti-hypertensives to attain BP control and adequate proteinuria often coexisted in children suffering from nephrotic syndrome. WHAT IS KNOWN: ⢠Anemia is a significant complication in children suffering from nephrotic syndrome. ⢠Cause of anemia in nephrotic syndrome is multifactorial. WHAT IS NEW: ⢠Iron deficiency anemia was the most common cause of anemia in Indian children with nephrotic syndrome. ⢠Anemia and need for anti-hypertensives to attain adequate BP control and proteinuria often coexisted in children with nephrotic syndrome.
Assuntos
Anemia Ferropriva , Anemia , Síndrome Nefrótica , Criança , Humanos , Síndrome Nefrótica/complicações , Síndrome Nefrótica/tratamento farmacológico , Anti-Hipertensivos/uso terapêutico , Estudos Prospectivos , Anemia/epidemiologia , Anemia/etiologia , Proteinúria/complicações , Esteroides/uso terapêuticoRESUMO
BACKGROUND AND OBJECTIVES: Blood donor variability can affect the storage properties of packed red blood cells (PRBCs). This study aimed to determine the association of donor characteristics with in vitro storage haemolysis of PRBCs. MATERIALS AND METHODS: In the prospective observational study, a total of 109 whole blood donors were enrolled using the purposive sampling method. A pre-donation sample was collected for haemoglobin (Hb) and serum uric acid (UA) levels. PRBC aliquots were tested for potassium, lactate dehydrogenase (LDH), Hb, haematocrit, plasma Hb and haemolysis on days 1, 21 and 35 of storage. The association of these parameters with donor age, sex, donation status, dietary pattern and body mass index was determined. RESULTS: Mean haemolysis was significantly higher in PRBCs from donors with UA levels ≤6 mg/dL than donors with UA levels >6 mg/dL on day 35 of storage (0.22 ± 0.11 vs. 0.18 ± 0.07, p = 0.03). Median plasma Hb (mg/L) was significantly higher in PRBCs from first-time donors on day 21 (586 vs. 509, p = 0.05) and day 35 (1507 vs. 1358, p = 0.02) of storage in comparison to frequent donors. Significantly higher mean potassium (p = 0.04 day 1; p = 0.02 day 21) and median LDH values (p = 0.02 day 1, p = 0.05 day 21) were observed in PRBCs from male donors. A statistically significant positive association was observed between donor UA and LDH levels of PRBCs on day 35 of storage (ß coefficient: 715.52, p-value: 0.003) on multiple regression analysis. CONCLUSION: In vitro haemolysis of PRBCs is affected by blood donor characteristics.
RESUMO
Human brucellosis is a common zoonotic disease in certain regions of the world, especially in rural populations, yet it is often neglected. The index patient presented with prolonged fever and headache. Examination revealed hepatosplenomegaly, and investigations revealed transaminitis with bicytopenia with serological evidence positive for Brucella. The radiological picture suggested granulomatous disease with miliary nodules, and a bone marrow examination revealed a noncaseating granuloma. The case was unique and challenging, as the clinical picture was consistent with epidemiologically prevailing tuberculosis, which is also seen in brucellosis-endemic countries. Brucellosis mimicked disseminated tuberculosis, making the case intriguing to diagnose and treat. The case demonstrated that physicians need to have a high index of suspicion for brucellosis, which should be actively investigated in cases with prolonged fever. It also illustrated the fundamental importance of teamwork and the centrality of the multidisciplinary team in the planning and prioritization of treatment.
Assuntos
Brucella , Brucelose , Tuberculose , Animais , Humanos , Brucelose/diagnóstico , Brucelose/tratamento farmacológico , Zoonoses , Tuberculose/diagnóstico , Tuberculose/tratamento farmacológico , Febre , GranulomaRESUMO
Background Angiogenesis plays a key role in the development, maintenance, and progression of tumor. The incidence of non-Hodgkin's lymphoma (NHL) is increasing from the past three decades. Materials and Methods The aim of the study is to evaluate microvessel density (MVD) using CD34 monoclonal antibody and vascular endothelial growth factor (VEGF) using monoclonal antibody that were studied in pretreatment paraffin-embedded tissue samples of 60 cases. Results MVD was found to be increased in parallel with increasing grade of tumor. B-NHL had a mean MVD of 79.5 ± 8.8 (no./mm 2 ), while T-NHL had a mean MVD of 183 ± 37.6 (no./mm 2 ). VEGF expression was seen in 42 cases (70%), 20 cases (33.3%) showed strong VEGF expression, and the remainder showed either weak (36.6%) or no (30%) staining. Strong VEGF expression is seen in 100% cases of T-NHL and 77.7% cases of B-NHL. Mean MVD and VEGF expression was found to be correlated significantly with the histological grade of NHL ( p = 0.001 and p = 0.000, respectively). Average microvessel counts were 53, 82.9, and 130.8 vessels (no./mm 2 ) for negative, weak, and strong VEGF staining, respectively. These differences were statistically significant ( p = 0.005 for strong vs. negative and p = 0.091 for strong vs. weak VEGF staining individually). Conclusion As the grade of tumor progresses, the angiogenic potential also advances which seems to depend on VEGF. The presence of higher MVD in high-grade lymphomas can be utilized for antiangiogenic drugs.
RESUMO
OBJECTIVES: Therapeutic phlebotomy allows for a controlled and gradual decrease in red cell mass leading to improved blood flow and symptomatic relief in polycythaemia. The present study was aimed to determine the impact of serial fixed volume and fixed interval therapeutic phlebotomy protocol on the laboratory and clinical parameters in patients of polycythaemia. MATERIAL AND METHODS: This prospective longitudinal study was conducted over 18 months. The desired haematocrit for polycythemia vera and secondary polycythemia was 45% and 52% respectively. A fixed volume of 350 ml phlebotomy was performed every-three days till the achievement of desired haematocrit. Complete blood count was performed before and after each procedure and iron studies were done at the time of enrolment and after the achievement of desired haematocrit. Post-procedure symptomatic relief was assessed by a 10-point visual analogue scale (VAS). RESULTS: Of the 29 patients enrolled in the study, 3 patients were lost to follow up and data of 26 patients was analyzed. Mean Hb declined from 17.84 ± 1.88 gdL-1 to 14.67 ± 1.14 gdL-1 (p < 0.001) and mean haematocrit decreased from a baseline of 57.11 ± 5.47% to 46.27 ± 3.763% (p < 0.001) upon achievement of desired haematocrit. There was a significant decline in serum iron from the baseline of 132.85 ± 94.136 µg dL-1 to 69.41 ± 58.643 µg dL-1 at desired haematocrit. A significant change in VAS score of almost all clinical parameters was observed. Post phlebotomy hematocrit correlated negatively with the number of procedures (p = 0.015). CONCLUSION: Our protocol yielded rapid and marked improvement in patients of primary and secondary polycythemia with minimal adverse events and significant amelioration of clinical parameters.
Assuntos
Policitemia Vera , Policitemia , Humanos , Policitemia/etiologia , Policitemia/terapia , Flebotomia , Estudos Longitudinais , Estudos Prospectivos , Policitemia Vera/terapia , Policitemia Vera/complicações , Hematócrito/métodosRESUMO
INTRODUCTION: Type 2 diabetes mellitus (T2DM) is a metabolic disorder associated with vascular complications that are attributable to dysregulated platelet reactivity as measured by mean platelet volume (MPV). This study aimed at determining a relationship between MPV and glycaemic control in new-onset T2DM. METHODS: This was a prospective study conducted on 236 new-onset T2DM patients divided in two groups as group A, glycosylated haemoglobin A1c (HbA1c < 7.9%; n = 70) and group B, HbA1c ⩾ 8% (n = 107) who were followed up for 6 months for change in platelet and glycaemic parameters. RESULTS: At 6-month follow-up, there was a significant decrease in HbA1c and MPV (group A (HbA1c: 7.40 ± 0.40 vs 7.03 ± 0.23%, p < 0.03; MPV: 9.65 fl ± 0.74 vs 9.46 fl ± 0.71, p < 0.001) and group B (HbA1c: 10.59 ± 1.89% vs 9.29 ± 1.50%, p < 0.001; MPV: 10.89 fL ± 1.29 vs. 10.23 fL ± 1.40, p< 0.001)). The percentage decline in HbA1c was more in group B (11.81 ± 5.87 vs 4.76 ± 4.58, p < 0.01). There was a positive correlation between ΔMPV and ΔHbA1c in group B; however, we did not observe significant correlation for group A. CONCLUSION: We interpret that in people with diabetes with baseline HbA1c ⩾ 8%, with improvement in glycaemic control, there is a significant decrease in MPV. We propose that a routine MPV testing can be used as a potential marker for glycaemic control in T2DM.
Assuntos
Diabetes Mellitus Tipo 2 , Volume Plaquetário Médio , Diabetes Mellitus Tipo 2/complicações , Hemoglobinas Glicadas/análise , Hemoglobinas Glicadas/metabolismo , Controle Glicêmico , Humanos , Estudos ProspectivosRESUMO
Objectives Hypocellular bone marrow (BM) disorders comprise heterogeneous entities associated with peripheral cytopenias and decreased production of hematopoietic cells in BM. This study was undertaken to analyze immunohistochemical expression of CD34, CD117, and p53 in morphologically diagnosed patients of hypocellular BM (aplastic anemia [AA], hypocellular myelodysplastic syndrome [h-MDS], and hypocellular acute myeloid leukemia [h-AML]). Materials and Methods BM specimens were obtained from patients presenting with pancytopenia/bicytopenia. On 30 patients diagnosed as hypocellular BM, immunohistochemistry (IHC) for CD34, CD117, and p53 was performed. Results BM cellularity was < 30% in all (100%) patients. Blast count was increased in h-MDS and h-AML. Features of dysplasia were noted in six (20%) patients. Out of these, three patients were diagnosed as h-MDS having bilineage/trilineage dysplasia, and the other three patients were of AA (11.5% patients) displaying only dyserythropoiesis. On IHC, percentage of BM CD34+ cells was increased in h-MDS+ h-AML (3.87 ± 0.86) as compared with AA (0.19 ± 0.15) and controls (0.81 ± 0.21), p = 0.01. Percentage of BM p53+ cells was also increased in h-MDS+ h-AML (2.9 ± 2.07) as compared with AA and controls, which did not show any p53+ cells, p = 0.0. No statistically significant difference was observed in the expression of CD117 in h-MDS+ h-AML (4.95 ± 3.40) compared with AA (4.49 ± 1.07), p = 0.99. Conclusion The study demonstrates the usefulness of CD34 and p53 immunoexpression as an important ancillary method in distinguishing various hypocellular BM disorders, especially h-MDS and AA. However, the role of CD117 remains unclear and needs to be evaluated further by larger studies.
RESUMO
BACKGROUND: Iron deficiency anaemia is the most common nutritional deficiency disorder in the world. Iron deficiency is a potential complication in repeated apheresis donation. The present study was aimed to evaluate serum iron stores in regular plateletpheresis donors. MATERIALS AND METHODS: A total of 60 donors were included in this study, which included 30 regular plateletpheresis donors as cases and controls were 30 first time donors. The donor samples were collected before donation for complete hemogram, transfusion transmissible infections screening and serum iron, total iron binding capacity, percentage saturation of transferrin and serum ferritin. RESULTS: Out of 60 donors, more than half of the donors (56.6 %) had serum ferritin less than 30 ng/mL. Out of these 34 donors, 25 were from the case group and 9 donors in the control group. The median serum ferritin level in cases and controls was 11.86 ng/mL (Interquartile range 4.18-17.34 ng/mL) and 37.92 ng/mL (Interquartile range 27.87-86.20 ng/mL) respectively (p < 0.001). The mean serum iron in cases and controls was 71.23 ± 31.32 µg/dL and 93.53 ± 33.53 µg/dL respectively (p = 0.016). The mean percentage saturation in cases and controls was 20.09 ± 9.31 % and 26.26 ± 9.03 % respectively (p = 0.012). A significant decline in mean serum ferritin with increase in number of annual donations and decrease in donation interval was observed. DISCUSSION: Regular plateletpheresis donation may lead to depletion of iron stores and subclinical iron deficiency. Donors with high platelet count are more likely to exhibit iron deficiency. Periodic serum ferritin estimation in donors participating in regular plateletpheresis donation is warranted.
Assuntos
Doadores de Sangue/estatística & dados numéricos , Deficiências de Ferro/etiologia , Ferro/sangue , Plaquetoferese/métodos , Adolescente , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Estudos Prospectivos , Adulto JovemRESUMO
BACKGROUND: Blood donors are prone to have iron deficiency. The aim of this study was to determine utility of serum hepcidin as an indicator of iron deficiency in blood donors. METHODS: A total of 200 voluntary, healthy blood donors were included in the study. Donors were categorized into four groups according to the donation frequency. Group I: (n = 50) who donated for the first time, or those who have not donated in the past 2 years (reactivated donors). Group II: (n = 50), who donated blood for the second time and had donated once in the previous 12 months. Group III: (n = 50), who donated blood for third time and had donated twice in the previous 12 months. Group IV: (n = 50) who donated blood for the fourth time and had donated thrice in the previous 12 months. Sera of study participants were evaluated for serum ferritin and serum hepcidin levels based on enzyme linked immunosorbent assay. RESULTS: Serum hepcidin concentration ranged 2.36-15734 pg/mL. Serum hepcidin and serum ferritin were found to be lowest in group IV donors. When serum ferritin concentration of less than 15 ng/mL was considered as gold standard for diagnosing iron deficiency, AUCROC for serum hepcidin as a diagnostic test of iron deficiency was found to be 0.715. Serum hepcidin showed statistical significant correlation with donation frequency(p = 0 .005) and serum ferritin (p = 0.01). Sensitivity and specificity of serum hepcidin was found to be 77.8 %, 79.6 % respectively. CONCLUSION: Effectiveness of Serum hepcidin as a diagnostic marker of iron deficiency still needs to be determined.
Assuntos
Hepcidinas/metabolismo , Deficiências de Ferro/diagnóstico , Adulto , Doadores de Sangue , Feminino , Voluntários Saudáveis , Humanos , Deficiências de Ferro/patologia , Masculino , Estudos ProspectivosRESUMO
We present a family who suffered recurrent sibling losses due to vitamin K deficiency bleed. The index child was asymptomatic at presentation, had normal clinical examination, and was investigated for coagulation disorders in view of previous 3 sibling losses as a result of intracranial hemorrhage. His investigations showed deranged coagulogram and clotting factors' assay. The baby was given vitamin K1 1 mg intramuscularly following which his coagulogram and clotting factors' assay returned to normal. The genetic analysis did not identify any inherited cause of bleeding tendency. The significant family history, exclusive breastfeeding, no diarrhea, failure to thrive or drug use, no prophylaxis with vitamin K at birth, recovery of clotting factors on vitamin K administration, and a corroborative molecular analysis confirmed diagnosis of vitamin K deficiency in the index child. This case gives a strong reminder not to miss birth dose of vitamin K in any neonate.
Assuntos
Antifibrinolíticos/uso terapêutico , Hemorragias Intracranianas/tratamento farmacológico , Deficiência de Vitamina K/tratamento farmacológico , Vitamina K/uso terapêutico , Coagulação Sanguínea/efeitos dos fármacos , Humanos , Lactente , Recém-Nascido , Hemorragias Intracranianas/sangue , Hemorragias Intracranianas/etiologia , Masculino , Irmãos , Deficiência de Vitamina K/sangue , Deficiência de Vitamina K/complicaçõesRESUMO
Bone marrow suppression has a wide variety of causes. One of the overlooked causes is linezolid, a drug that is now being extensively used in the management of not only soft tissue infections but also hospital-acquired infections. Methicillin-resistant Staphylococcus aureus (MRSA) is widely being treated with linezolid. It becomes imperative that we comprehensively understand the hematological adverse effect profile of this drug. A reversible myelosuppression is seen with its extended use, though a number of risk factors like renal impairment are usually present. A prompt diagnosis can help us to timely discontinue the drug. We report one such case of an elderly patient with septic arthritis of the knee who developed pancytopenia after 32 days of linezolid therapy. Withdrawal of the drug led to a complete recovery of the blood counts in 21 days.
RESUMO
Sea blue histiocytosis is an unusual bone marrow finding in many haematological conditions or lipid metabolic diseases that by itself may not carry any prognostic value. It may occur rarely as a primary genetic clinical syndrome characterized by splenomegaly, hypertriglyceridemia and thrombocytopenia. More commonly, the presence of these lipid-laden blue-stained macrophages indicates an underlying condition characterized by increased bone marrow precursor cell turnover due to myeloproliferative conditions or ineffective erythropoiesis. Rarely may they be observed in cases of immune thrombocytopenic purpura (ITP) incidentally due to rapid megakaryocytic turnover. Sea blue histiocytosis should prompt the clinician to evaluate the patient for more sinister conditions such as myelodysplastic syndrome or infiltrative disorders.
RESUMO
Background Multiple myeloma (MM) is a plasma cell disorder characterized by monoclonal proliferation of plasma cells in bone marrow. Plasmablastic MM is a morphologic subset of MM, containing ≥2% plasmablasts of all plasma cells. Methods The study included 30 consecutively diagnosed patients of MM (6 plasmablastic, 24 nonplasmablastic) over a span of 2 years. Angiogenesis in MM was assessed by analysis of vascular endothelial growth factor (VEGF) immunoexpression by plasma cells and microvessel density (MVD) using anti-CD34 antibody. CD34 and VEGF immunohistochemical staining was performed in all the 30 cases. Angiogenesis was studied in relation to plasmablastic morphology and clinical profile to determine if any correlation exists between these. Results The mean VEGF expression of 80.83 ± 7.36 in plasmablastic myeloma cases was significantly higher compared with a mean VEGF of 53.54 ±17.09 in nonplasmablastic cases. Most of the cases (66.6%) of plasmablastic myeloma exhibited strong (3+) VEGF expression. The difference in mean VEGF expression between plasmablastic and nonplasmablastic cases was found to be statistically significant ( p = 0.001). The mean MVD in plasmablastic cases was 44.8 ± 3.69, while in the nonplasmablastic category, the mean MVD was 23.7 ± 5.14, difference being statistically significant ( p < 0.05). Also, a positive correlation was found between VEGF expression and MVD. Conclusion A moderate/strong VEGF intensity and higher MVD were found in cases of plasmablastic MM, suggesting that a more aggressive histological disease may be associated with increased production of VEGF. This finding might be helpful to identify a subset of patients with adverse prognosis and to provide antiangiogenic therapy to improve their survival. However, studies comprising larger number of patients are required to bring out a statistical significance to further substantiate these findings.
RESUMO
Disseminated tuberculosis (DTB) often presents with protean clinical manifestations that often leads to potential diagnostic dilemmas. The nonspecific features may include pyrexia of unknown origin, hepatosplenomegaly, lymphadenopathy, meningitis, and a variety of hematological abnormalities, namely anemia, pancytopenia, and leukemoid reaction. Tuberculosis is one of the nonhematopoietic diseases that has been reported in conjunction with myelofibrosis. We, hereby, report a case of DTB with massive splenomegaly, severe pancytopenia, and marrow fibrosis.
Assuntos
Mielofibrose Primária/diagnóstico , Mielofibrose Primária/microbiologia , Tuberculose Miliar/diagnóstico por imagem , Antituberculosos/uso terapêutico , Medula Óssea/microbiologia , Medula Óssea/patologia , Diagnóstico Diferencial , Evolução Fatal , Humanos , Masculino , Pessoa de Meia-Idade , Pancitopenia/microbiologia , Mielofibrose Primária/tratamento farmacológico , Esplenomegalia/microbiologia , Tomografia Computadorizada por Raios X , Tuberculose Miliar/sangue , Tuberculose Miliar/tratamento farmacológicoRESUMO
Cardiac involvement in multiple myeloma is rare. We report a rapidly progressive atrial mass and plasma cell-infiltrated pericardial effusion with tamponade. Bone marrow biopsy and plasma immunoelectrophoresis confirmed multiple myeloma (Revised International Staging System Stage III). The patient died within 18 weeks of presentation, suggesting aggressive disease with poor prognosis. (Level of Difficulty: Beginner.).
RESUMO
Amyloidosis is a well-recognized entity, that can present as a systemic disease, or, uncommonly, as a localized mass. We report here, a rare presentation of amyloidosis presenting as a supraclavicular swelling, diagnosed on fine-needle aspiration cytology. Subsequent bone marrow examination led to the diagnosis of multiple myeloma. This case highlights the need to be aware of cytological characteristics of amyloidosis, which can have unusual presentation posing a diagnostic dilemma.
Assuntos
Amiloidose , Medula Óssea , Edema , Mieloma Múltiplo , Amiloidose/diagnóstico , Amiloidose/metabolismo , Amiloidose/patologia , Biópsia por Agulha Fina , Medula Óssea/metabolismo , Medula Óssea/patologia , Diagnóstico Diferencial , Edema/diagnóstico , Edema/metabolismo , Edema/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/metabolismo , Mieloma Múltiplo/patologiaRESUMO
Immune thrombocytopenia is an autoimmune disorder characterised by autoantibody production against platelets, increased platelet destruction and impaired thrombopoiesis. Steroids are the first-line agents whenever treatment is indicated; however, some patients may not respond and the responders may as well relapse while the dose is being tapered. Side effects of steroids prohibits their long-term use and patients often have to be switched to other agents. Standard drug management with intravenous immunoglobulins and thrombopoietin receptor analogues is difficult to administer in patients from low socioeconomic regions of the world making the management even more challenging. Hence, after reviewing the literature and considering the cost in comparison to all the second-line agents available, we tried dapsone in a steroid-dependent patient of immune thrombocytopenic purpura who had developed major steroid-related side effects. Patient showed good response to dapsone and has been in remission for around one and a half years.