scholarly journals Myeloproliferative Neoplasm with Prominent Eosinophilia

2014 ◽  
Vol 31 (3) ◽  
pp. 162-167
Author(s):  
S Giti ◽  
MN Bhuiyan ◽  
MR Hossain ◽  
MS Islam ◽  
F Ahmed ◽  
...  

The patient, a young soldier aged 36 years having past history of malaria, was admitted in CMH Dhaka on 17 August 2011 as a transferred case from CMH Saidpur and died on 22 August 2011. The deceased was admitted in CMH Saidpur on 05 August 2011 with high fever for 05 days along with generalized joint and muscle pain. In spite of all available treatment the patient was deteriorating and he was then transferred to CMH Dhaka. At that time the patient was febrile, dehydrated and toxic with lymphadenopathy, extremely tender joints and muscles. The patient rapidly developed acute kidney failure and gradually developed features of DIC. His bone marrow examination revealed dyserythropoiesis with predominantly eosinophilic granulopoiesis, suggestive of myeloproliferative neoplasm with prominent eosinophilia. The patient was treated with injectable antibiotics, antimalarial and oral prednisolone with all intensive care facilities. Ultimately all attempts were proved unsuccessful and he died on 22 August 2011 at 1700 hrs. On autopsy the deceased had intra atrial thrombus and possibly that was the immediate cause of death. A haematological malignancy, myeloproliferative neoplasm with prominent eosinophilia, can very well produce such a fatal condition. DOI: http://dx.doi.org/10.3329/jbcps.v31i3.20985 J Bangladesh Coll Phys Surg 2013; 31: 162-167

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3703-3703
Author(s):  
Kazuhiko Ikeda ◽  
Tsutomu Shichishima ◽  
Kazuko Akutsu ◽  
Yukio Maruyama

Abstract PNH is an acquired hematologic disorder which is characterized by complement-mediated hemolysis, thrombosis, and bone marrow failure. Also, PNH is one disorder of bone marrow failure syndromes, including aplastic anemia (AA) and myelodysplastic syndrome (MDS). It is well known that immunologic mechanisms by cytotoxic T lymphocytes (CTLs) contribute to pathophysiology of these disorders. In fact, some reports (Maciejewski et al, Blood, 2001; Shichishima et al, Blood, 2002) showed that HLA-DR*1501 is related with clinical pathophysiology of PNH. In this study, to clarify significance of CD8+ CTLs in pathophysiology of PNH, we investigated HLA class I (A and B) alleles in Japanese patients with PNH (female: male=7:17), AA (female: male=14:15), and MDS (female: male=6:16) by high-resolution method using polymerase-chain reaction after obtaining informed consent and approval from the institutional Human Research Committee. Mean age ± standard deviation of PNH, AA, and MDS patients was 52 ± 16, 54 ± 20, and 59 ± 18, respectively. HLA genotyping showed that the frequency of HLA-A*0206 allele in PNH patients (22.9%) was significantly different from those in 309 unrelated Japanese individuals (Saito et al, Tissue Antigens, 2000) (7.7%; p<0.02) and AA patients (5.2%; p<0.01). In contrast, we found no significant differences in the frequencies of the other alleles between PNH, AA, or MDS patients and the controls or between these disorders, except for high frequency of HLA-B40 alleles in AA patients (Nakamura et al, Blood, 2003). Then, various clinical parameters, including peripheral blood, bone marrow blood, and laboratory findings, proportions of glycosylphosphatidylinositol protein-negative population in erythrocytes, granulocytes, and monocytes, findings of chromosomal analyses and HLA-DR alleles, transfusion requirements, past history of AA, and history of thrombosis, were statistically compared between HLA-A*0206-positive group (n=10) and -negative group (n=14) in PNH patients. Statistical analyses showed that the reticulocyte counts , the values of lactate dehydrogenase, and the frequency of PNH patients with over 30% of CD59− erythrocytes in HLA-A*0206-positive group were significantly higher than in HLA-A*0206-negative group (121 ± 49 x 109/L vs 76.9 ± 43.9 x 109/L, p<0.03; 2866 ± 2606 IU/L vs 938 ± 775, p<0.02; and 80.0 % vs 28.6 %, p<0.02, respectively). Moreover, we found no AA (n=3) and MDS (n=5) patients with both the HLA-A*0206 allele and more than 1% of CD59− granulocytes. In conclusion, our findings suggest that the HLA-A*0206 allele in PNH may be correlated with the grade of the disease by complement-mediated hemolysis during negative selection of PNH clones, probably due to immunologic mechanisms by CD8+ CTLs.


2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
Michael Gilbertson ◽  
Annabel Tuckfield ◽  
Surender Juneja

We present the case of a 70-year-old woman who had a bone marrow examination performed to investigate marked thrombocytopenia in the context of a recent history of metastatic glucagonoma. Surprisingly this identified marked dysmegakaryopoiesis and fulfilled diagnostic criteria for refractory cytopenia with multilineage dysplasia, with a relatively uncommon associated cytogenetic lesion t(1;7). We present the case and review the literature of this cytogenetic lesion.


2018 ◽  
Vol 18 (6) ◽  
pp. 497-500
Author(s):  
Naveed Malek ◽  
Dominic G O’Donovan ◽  
Hadi Manji

An elderly Caucasian man presented with a 10-month history of proximal myopathy and dysphagia. His serum creatine kinase (CK) was elevated at 877 U/L (normal 40–320) and electromyography confirmed a myopathic process. Blood and urine tests suggested myeloma; bone marrow examination showed 30% plasma cells and stained positive for amyloid. The muscle biopsy was initially reported as normal but in the light of the bone marrow report, the biopsy specimen was stained for amyloid, which was positive. We diagnosed systemic amyloidosis causing a myopathy and have started treatment for myeloma.


2012 ◽  
Vol 9 (75) ◽  
pp. 2639-2652 ◽  
Author(s):  
Ian M. Hall ◽  
Iain Barrass ◽  
Steve Leach ◽  
Didier Pittet ◽  
Stéphane Hugonnet

Intensive care units (ICUs) play an important role in the epidemiology of methicillin-resistant Staphyloccocus aureus (MRSA). Although successful interventions are multi-modal, the relative efficacy of single measures remains unknown. We developed a discrete time, individual-based, stochastic mathematical model calibrated on cross-transmission observed through prospective surveillance to explore the transmission dynamics of MRSA in a medical ICU. Most input parameters were derived from locally acquired data. After fitting the model to the 46 observed cross-transmission events and performing sensitivity analysis, several screening and isolation policies were evaluated by simulating the number of cross-transmissions and isolation-days. The number of all cross-transmission events increased from 54 to 72 if only patients with a past history of MRSA colonization are screened and isolated at admission, to 75 if isolation is put in place only after the results of the admission screening become available, to 82 in the absence of admission screening and with a similar reactive isolation policy, and to 95 when no isolation policy is in place. The method used (culture or polymerase chain reaction) for admission screening had no impact on the number of cross-transmissions. Systematic regular screening during ICU stay provides no added-value, but aggressive admission screening and isolation effectively reduce the number of cross-transmissions. Critically, colonized healthcare workers may play an important role in MRSA transmission and their screening should be reinforced.


2016 ◽  
Vol 35 (3) ◽  
pp. 283-286
Author(s):  
Shubhankar Mishra ◽  
Sunil Kumar Agarwalla ◽  
Sushree Smita Behura ◽  
Gouranga Charan Pattnaik

Familial hemophagocytic lymphohistiocytosis (FHLH) is a rare genetic disorder associated with early onset in life with overwhelming activation of T lymphocytes and macrophages invariably leading to death. We present two cases of FHLH admitted to our hospital at different points of time. First child presented with multiple episodes of GTCS and high grade fever. There was a history of sibling death before. He was having hepatosplenomegaly with leucopenia, hyper-triglyceridemia, hyper-ferritinemia and bone marrow revealed abundant hemophagocytes in smear. Second case was a 6 month male with complaint of (Generalised Tonic Clonic Seizure (GTCS) with past history of repeated attacks of acute Respiratory Infection and neuroinfection. Previous sibling died in similar presentation. He was having hepatosplenomegaly, leucopenia, hyper triglyceridemia, hyper ferritinemia with abundant hemophagocytes in bone marrow smear. Both the cases were diagnosed as FHLH and treated according to protocol.Nepal Paediatr Soc 2015;35(3):283-286


2019 ◽  
Vol 2019 ◽  
pp. 1-9
Author(s):  
Osama M. Wassif ◽  
Abdo S. Abdo ◽  
Mona A. Elawady ◽  
Abeer E. Abd Elmaksoud ◽  
Rasha Sh. Eldesouky

Postpartum depression (PPD) is a mood disorder that begins after childbirth and usually lasts beyond six weeks; depression is often comorbid with anxiety. The main objectives of this work were to measure the prevalence of postpartum depression and/or anxiety among females in the Qaliubeya governorate to explore the underlying factors of these disorders and find if progesterone level has a role. A crosssectional study was conducted upon 500 postpartum females attending primary health care facilities in the Qaliubeya governorate. Data were collected by an interview questionnaire which included data about sociodemographic, obstetric, and past history and the Arabic version of DASS for assessment of postpartum depression and/or anxiety. The results showed 1.6% of the studied females suffered postpartum depression alone, 10% suffered from anxiety alone, and 21.2% suffered from both. The mean age of female who suffered from comorbid depression and anxiety was significantly (p=0.01) higher than the normal group (26.9 and 25.1, respectively), and they had a significantly lower socioeconomic score than the normal ones (31.1 and 34.1, respectively), p<0.05. There was a significant association (p<0.001) between the past history of similar conditions and the current prevalence of postpartum disorders. ROC curve analysis showed that the progesterone level ≤4.6, ≤11.3, and ≤2.8 significantly predict depression alone, anxiety alone, and comorbid diseases, respectively. It was concluded that postpartum depression and/or anxiety affect 32.8% of females in the Qaliubeya governorate. Very low socioeconomic level, lower educational levels, past history of similar conditions, and low progesterone level are the significant predictors.


Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4278-4278
Author(s):  
Andrew Jack ◽  
Daniel Painter ◽  
Alexandra Smith ◽  
Eve Roman ◽  
Ruth M de Tute ◽  
...  

Abstract Bone marrow examination is an established component of the process used to stage patients with diffuse large B-cell lymphoma (DLBCL), follicular lymphoma (FL) and classical Hodgkin Lymphoma (CHL); and it is a general requirement of clinical trials. The procedure involves significant discomfort and inconvenience to the patient, as well as expense for the NHS, and this study was designed to evaluate the clinical utility of the information derived from staging bone marrow examinations. All patients presenting with DLBCL (n=1908), CHL (n=604) and FL (n=770) between 1st Sept 2004 and 31st August 2011 in the UK's population-based (3.6M) study area (Haematological Malignancy Research Network - www.hmrn.org) were included. Comprehensive clinical data, including diagnostic, prognostic, outcome and socio-demographic information are available for all patients. All diagnostic and staging specimens are reported in a single specialist haematopathology laboratory (the Haematological Malignancy Diagnostic Service – www.hmds.info). HMDS routinely undertake morphology and flow testing on bone marrow aspirate and trephine biopsies; while further molecular and cytogenetic tests are carried out at clinical discretion. This study aimed to assess the impact of bone marrow examination on the calculation of disease specific prognostic indices. Bone marrow examinations were performed in 85% of patients, providing morphological evidence of disease in 10.2% of DLBCL, 7.2% of CHL and 36.5% of FL. In 18 patients with DLBCL and 16 patients with CHL the bone marrow was the presenting site of disease and this was associated with a very poor clinical outcome. In DLBCL knowledge of the bone marrow result increased the IPI by 1 point in 4.0% and by 2 points in 1.1% of cases. Similarly, the Hasenclaver index increased by 1 point in 3.6% of CHLs and the FLIPI increased by 1 point in 9.0% of FLs. In the case of DLBCL, 70% of these patients had an IPI score of >2 before the results of the marrow examination were added. As well as the assessment of prognosis, the results of bone marrow examination are a component of a number of key clinical decisions. Patients with stage 1 FL are routinely treated with radiotherapy with curative intent. In the absence of a bone marrow result, an additional 27 patients would have potentially been treated with radiotherapy. The 5 year relative survival (corrected for underlying population mortality rates) of this group was similar to those with stage 2 disease on watch and wait, and was slightly inferior to that seen in true stage 1 disease. Similarly, patients with stage1A DLBCL may be treated with 3 courses of R-CHOP and radiotherapy in preference to 6 or 8 courses of R-CHOP alone. In our data , lack of knowledge of the bone marrow result would not have affected the classification of this group in. In DLBCL the decision to give prophylaxis to prevent CNS relapse may be based on 2 or more extranodal sites, including bone marrow, involved at presentation. In this group, this decision could have been affected in 51 cases. In the UK the approximate cost of taking and reporting a bone marrow is around $900. When used routinely the cost could approach $25,000 per patient whose IPI score is increased by the results of examination; the figure for CHL is $44,000. It is, therefore, difficult to justify the cost effectiveness of this approach. In newly presenting patients with lymphoma, bone marrow examination should be reserved for those with unexplained cytopenia, and those who may potentially require radiation therapy for early stage FL or CNS prophylaxis in DLBCL. Disclosures: Jack: Roche /Genentech: Research Funding. Off Label Use: Rituximab in Burkitts Lymphoma. Patmore:Roche: Consultancy, Honoraria.


2015 ◽  
Vol 2 (1) ◽  
pp. 25-27
Author(s):  
Subhash Acharya ◽  
Sabin Koirala ◽  
Anjan Shrestha ◽  
Diptesh Aryal

An elderly female presented to Intensive Care Unit with generalized weakness and altered sensorium with past history of adenocarcinoma of larynx. She was managed with Radiation Therapy and Chemotherapy in the past. Current admission to the intensive care unit was for aspiration pneumonitis and respiratory failure requiring mechanical ventilation. She then progressively developed muscle weakness, which was provisionally diagnosed and managed as critical illness polyneuromyopathy (ICU acquired weakness) and muscle biopsy was sent, as there were no obvious neurological signs and she was not recovering from her muscle weakness as well. She then developed Catheter related sepsis and refractory septic shock, and then she passed away. Her muscle biopsy report that was available postmortem revealed that she had severe toxoplasma infestation that remained in shadow.Journal of Society of Anesthesiologists of Nepal 2015; 2(1): 25-27


2019 ◽  
Vol 31 (1) ◽  
pp. 15-18 ◽  
Author(s):  
Md Rezaul Karim Chowdhury ◽  
Md Haroon Ur Rashid ◽  
Amina Begum

Introduction: Bone marrow study has wide application in clinical medicine. It is important test not only for diagnosis of haematological diseases but also for various systemic illnesses. The aim of this study is to determine the indications, the spectrum of haemotological and non haematological disorders diagnosed by using this procedure. Materials & Methods: It was a prospective study comprising 152 patients who underwent bone marrow examination for evaluation of haematological and nonhaematological disorders in the Department of Haematology, Enam Medical College Hospital during the period of 2012 to 2017. Results: In our study male to female ratio was 1.6:1 and common age group was >45years (n-65, 42.76%). Most common indications for bone marrow examination were pancytopenia (26.97%, n-41) and diagnosis of leukaemia/myeloproliferative neoplasm (25.66%, n-38). 90.13% (n-137) marrows were pathological. Non-malignant conditions were 40.79% (n-62) and malignant conditions were 49.43% (n-75). Non malignant haematological condition were 33.55% (n-51), malignant haematological conditions were 47.37% (n-72). Most common nonmalignant haematological conditions were aplastic anaemia (15.13%, n-23) and immune thrombocytopenic purpura (9.87%, n-15). Visceral leishmaniasis was found 3.29% (n-5). Acute myelogenous leukaemia (14.47%, n-22) and multiple myloma (11.18%, n-17) were the most common malignant haematological condition. Secondary deposit was found 1.97% (n-3). Conclusion: Bone marrow examination is a simple invasive procedure for diagnosis of both haematological and nonhaematological diseases when routine investigations failed to reach the final diagnosis. Medicine Today 2019 Vol.31(1): 15-18


Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 385-385 ◽  
Author(s):  
Laurence A. Boxer ◽  
Audrey Anna Bolyard ◽  
Beate Schwinzer ◽  
Darryl Glaser ◽  
Kenneth Dougan ◽  
...  

Abstract Autoimmune Neutropenia of Infancy (ANI) is generally regarded as a benign condition diagnosed by finding neutropenia and a positive test for antineutrophil antibodies. To determine whether the presence of antineutrophil antibodies in infancy always predicts a benign disease, data were reviewed for young children with positive antineutrophil tests who are enrolled in the Severe Chronic Neutropenia International Registry (SCNIR). A set of identical twins presented with severe neutropenia at age 5 months. Twin A1 had a preceding history of aphthous ulcers, cellulitis, recurrent otitis media, pneumonitis, an episode of bacteremia and a positive test for antineutrophil antibodies&lt;INS cite=mailto:%20 dateTime=2005-08-01T10:53&gt;.&lt;/INS&gt; Twin A2 had a preceding history of cellulitis, otitis media, and pneumonitis. The median absolute neutrophil count (ANC) of twin A1 and twin A2 was 0.280 x 109/L and 0.116 x 109/L, respectfully and bone marrow examination for twin A1 showed maturation arrest at the myelocyte-metamyelocyte level. With the diagnosis of ANI in one twin, both twins were placed on granulocyte-colony stimulating factor (G-CSF), because of frequent fever and recurrent infections. Fifty-one months after the diagnosis of immune neutropenia, twin A1 developed acute myelogenous leukemia. Further studies then revealed that both twins had a mutation in the neutrophil elastase gene (ELA2) at exon 5 resulting in a nucleotide substitution from TGC to TGA, which indicated a diagnosis of severe congenital neutropenia. Another set of fraternal twins were identified to have ANI at six months because of recurrent abscesses, mouth ulcers, otitis media and positive tests for antineutrophil antibodies. The ANC was 0.067 x 109/L in twin B1 and 0.166 x 109/L in twin B2. At age 31 months twin B1 developed a severe Clostridial infection, which led to the loss of his right leg and part of the anterior abdominal wall. Subsequently both twins were placed on G-CSF. Prior to G-CSF treatment, bone marrow studies revealed an arrest at the promyelocyte stage and an ELA2 mutation in exon 4 leading to a TCG to TTG substitution in both twins thereby indicating a diagnosis of severe congenital neutropenia. Upon further review of the SCNIR, eight other congenital patients were identified to have antineutrophil antibodies and bone marrow patterns consistent with severe congenital neutropenia. In conclusion, it is important to be aware that positive antineutrophil antibodies can occur associated with severe congenital neutropenia. Infants with severe neutropenia and repeated or severe bacterial infections should have a bone marrow examination and ELA2 evaluation to establish correctly the underlying pathogenesis of the neutropenia.


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