Presentation
The purpose of this study is to present the evolution of such a case, with difficulties in establishing not only the correct diagnosis, but also the appropriate therapeutic approach. [ncbi.nlm.nih.gov]
[…] simulators (Increased blasts make these less likely) Other myelodysplastic disorders Refractory Anemia with Excess Blasts Acute Myelogenous Leukemia Blasts Blasts frequently ≥20% in peripheral blood and/or bone marrow AML defining translocations not present [surgpathcriteria.stanford.edu]
Generally, the patient presents with normochromic, normocytic anemia with overall erythroid hyperplasia.[1][2] Often, the level of hemoglobin is generally in the range of 9 to 12 g/dL, though lower levels may be present. [statpearls.com]
The number of megakaryocytes is also diminished and there are prominent micro-megakaryocytes present throughout. Iron stores are present and no ringed sideroblasts are seen. The bone marrow is >90% cellular. [gpec.ubc.ca]
Entire Body System
- Weakness
* Poor appetite * Irritability * Weakness * Death Causes - Refractory anemia Not supplied. [checkorphan.org]
If you have symptoms, they may include Shortness of breath Weakness or feeling tired Skin that is paler than usual Easy bruising or bleeding Pinpoint spots under the skin caused by bleeding Fever or frequent infections Myelodysplastic syndromes are rare [icdlist.com]
(effective October 01, 2015) D46.4 Refractory anemia, unspecified (effective October 01, 2015) Signs and Symptoms Easy bruising or bleeding Petechiae (flat, pinpoint spots under the skin caused by bleeding) Shortness of breath Skin paler than usual Weakness [seer.cancer.gov]
ADDITIONAL LABORATORY INVESTIGATIONS: Routine pretransfusion serological testing did not reveal the anti-A1 antibody (Table 1); however, when the serum:cell ratio of the pre-transfusion specimen was increased, a weak and 1+ reaction was seen at the immediate [path.upmc.edu]
Respiratoric
- Exertional Dyspnea
Case Report A 74-year-old African-American man was admitted to our institution with a three month history of progressive generalized weakness accompanied by a 20lb weight loss and progressive exertional dyspnea. [ijcasereportsandimages.com]
Workup
Scope of presentation Clinical presentation Diagnostic workup Management approach Choosing agent for treatment 3. [slideshare.net]
Workup may include: CBC with differential, liver function tests, renal function tests, blood and urine arsenic levels. Arsenic levels in tissue, hair and nails can be performed, but may be normal if only distant exposure. [wiydx.com]
Initial laboratory workup revealed an Hgb of 11.7 g/dl and MCV 102. The creatinine clearance was 40 milliliter per minute, serum ferritin 444 μg/L and percent transferrin saturation of 41. [omicsonline.org]
Treatment
A median treatment duration of 9 (range 1 to 28) months was required to achieve multilineage recovery. The responders showed an ability to maintain the multilineage recovery for 9+ to 47+ months and to tolerate long-term treatment. [ncbi.nlm.nih.gov]
Lenalidomide treatment was therefore stopped. During the treatment period her spleen had started to soften and over the following 2 months off treatment there was complete resolution of her palpable massive splenomegaly. [hindawi.com]
Overview of Treatment 1. [slideshare.net]
Prognosis
(Type 2 RAEB), the prognosis is around 10 months survival Proper care should be taken to treat and control complications from MDS. [dovemed.com]
The prognosis is poor with death usually occurring within a couple of years. Type 1 refers to cases where the level of blasts is less than 10% and type 2 refers to cases where the level of blasts is 10-20%. [checkorphan.org]
This case might interest pathologists, but especially clinicians, for at least two reasons: the rarity of this disease and the lack of data on prognosis of these patients, probably because of relatively recent established diagnosis criteria and existence [ncbi.nlm.nih.gov]
Etiology
Etiology The etiology is not known but is thought to involve inherited susceptibility or hematopoietic stem cell damage. Prognosis Median survival is reported to be 6 to 7 years and 5% to 10% of patients progress to acute leukemia. [orpha.net]
[…] increased blasts or Auer rods 2 categories based on prognosis RAEB-1 2-4% blasts in peripheral blood or 5-9% blasts in bone marrow RAEB-2 5-19% blasts in peripheral blood or 10-19% blasts in bone marrow Auer rods, if present, advance MDS case to RAEB-2 ETIOLOGY [basicmedicalkey.com]
Two etiologic categories of MDS: 1.) [slideplayer.com]
Four years ago, bone marrow aspiration was performed for anemia etiology with the prediagnosis of myelodysplasic syndrome but it did not reveal any pathological findings. [go.galegroup.com]
Well-organized Major Infectious Diseases chapters break down content by etiologic agent and epidemiology, clinical signs and their pathophysiology, physical examination findings, diagnosis, treatment and prognosis, immunity, prevention, and public health [books.google.es]
Epidemiology
Epidemiology and Clinical Features Epidemiology The epidemiologic features of refractory anemia with ring sideroblasts (RARS) are similar to those of most other types of myelodysplastic syndrome (MDS). [emedicine.medscape.com]
Summary Epidemiology Exact prevalence is unknown but RCUD accounts for about 10% to 20% of patients with MDS which has a prevalence estimated to be 1/25,000 to 1/33,000. The disease tends to occur in older adults with a male predominance. [orpha.net]
Infections in Compromised Hosts 593 Clinical Considerations 601 Approach to Patients With Systemic Infection 612 Epidemiology and Infection Control 621 PULIMONARY AND CRITICAL CARE 629 Respiratory Therapy Techniques and Mechanical 636 Management of Acute [books.google.com]
Epidemiology There are few data on the epidemiology of RA, which may account for 30-40% of all MDS cases. MDS is predominantly diagnosed in the elderly population. [atlasgeneticsoncology.org]
[…] blasts in bone marrow RAEB-2 5-19% blasts in peripheral blood or 10-19% blasts in bone marrow Auer rods, if present, advance MDS case to RAEB-2 ETIOLOGY/PATHOGENESIS Hematopoietic Stem Cell Disorder Clonal hematopoietic stem cell disorder CLINICAL ISSUES Epidemiology [basicmedicalkey.com]
Pathophysiology
Preisler, Michael Churchill, Abdullah Mahmood Ali, Azra Raza and Siddhartha Mukherjee DOI: 10.1158/1538-7445.AM2015-2425 Published August 2015 Proceedings: AACR 106th Annual Meeting 2015; April 18-22, 2015; Philadelphia, PA Abstract Background: The pathophysiology [cancerres.aacrjournals.org]
Well-organized Major Infectious Diseases chapters break down content by etiologic agent and epidemiology, clinical signs and their pathophysiology, physical examination findings, diagnosis, treatment and prognosis, immunity, prevention, and public health [books.google.es]
The precise pathophysiologic mechanism of ACD is unclear. The anemia is related more to decreased red blood cell production than to increased destruction. [aafp.org]
Although the pathophysiologic mechanisms underlying RARS are not entirely elucidated, there is compelling evidence of mitochondrial dysfunction in early stages of erythroid maturation, leading to abnormal mitochondrial iron deposition and ineffective [emedicine.medscape.com]
Several pathophysiological hypotheses may be raised: (i) toxic effects of one of the study drugs (olaparib or durvalumab), (ii) bone marrow invasion by tumour or (iii) parvovirus B19 infection. [gustaveroussy.fr]
Prevention
However, it was limited in terms of preventing leukemic transformation. Allo-SCT would seem to be the only available treatment that can alter disease progression. [ncbi.nlm.nih.gov]
Prevention Although there is a big genetic predisposition to MDS, some risk factors can be avoided to help prevent the disease. These include: Avoiding too much radiation exposure. [syndromespedia.com]
Prevention - Refractory anemia Not supplied. Diagnosis - Refractory anemia Not supplied. Prognosis - Refractory anemia Median survival of RA may fall in the 27-50 month range. [checkorphan.org]
The a priori hypothesis was that fluorouracil would be effective in preventing these cancers. [jamanetwork.com]