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Acute Erythremia

Erythroleukemia


Presentation

  • Poor-risk cytogenetics were present in 61% of the erythroleukemia patients and 38% of the control patients. Complete remission rates were 62% in the erythroleukemia group and 58% in the control group.[emedicine.com]
  • The most common cytogenetic abnormality was a complex karyotype, present in 49 patients (54%), including 40 (44%) patients who presented with abnormalities of chromosomes 5 and/or 7. Isolated trisomy 8 was present in 5.5% of cases.[doi.org]
  • […] at least 20% myeloblasts in the bone marrow Acute erythroid leukemia characterised by the presence of immature erythroid cells in the bone marrow (at least 80% of the cellular component), without evidence of a significant myeloblastic cell population present[icd9data.com]
Leg Swelling
  • Over the previous six months he had developed progressive tiredness, bilateral leg swelling, weight loss, and night sweats and, more recently, had noted epistaxis, haemoptysis, and central chest tightness at rest.[jcp.bmj.com]

Workup

  • Immunohistochemistry has a role in the workup of cases of AEL, particularly in cases in which the aspirate material is scant. Here we mention markers or applications not already addressed in the flow cytometry section above.[archivesofpathology.org]
Karyotype Abnormal
  • Acquired karyotypic abnormalities were found in 17 of 27 patients where analysis was attempted.[jcp.bmj.com]
  • Fourteen (88%) patients had bi- or trilineage and 13 (81.3%) patients had cytogenetic abnormalities including 11 patients with a complex karyotype. Abnormalities of chromosomes 5 and 7 were also common in patients with cytogenetic abnormalities.[nature.com]
Poikilocytosis
  • In 1988, dysplastic features (anisocytosis, poikilocytosis, tear drop poikilocytes, and occasional myelocytes and blasts) were noted in the peripheral blood. Bone marrow cytogenetic analysis was normal.[jcp.bmj.com]
  • Nonspecific red blood cell abnormalities can be present, such as anisocytosis, poikilocytosis, anisochromia, basophilic stippling, schistocytes, and erythrocytes that are poorly hemoglobinized.[archivesofpathology.org]
Normocytic Anemia
  • In this patient the smear showed hypochromic, normocytic anemia, leukopenia, and thrombocytopenia. No blasts were identified (Giemsa, original magnification 1000).[archivesofpathology.org]
Philadelphia Chromosome Positive
  • Philadelphia chromosome positive cases may be related to the blast crisis of CML. Extramedullary myeloid cell tumor (EMCT) or granulocytic sarcoma or chloroma is an extramedullary tissue mass of blasts and immature myeloid cells.[med-ed.virginia.edu]
Liver Biopsy
  • (F) A liver biopsy shows similar cells, immunoreactive for β-sialoglycoprotein, within hepatic sinusoids.[jcp.bmj.com]

Treatment

  • Problems encountered in the treatment of acute erythroleukemia include primary induction failure, relapse, and the toxicity of chemotherapeutic agents.[emedicine.com]
  • No treatment group was predictive of worse DFS by multivariate analysis.[doi.org]
  • Prognosis is worse in elderly patients, those with a history of myelodysplastic syndrome, and in patients who had previously received chemotherapy for the treatment of a different neoplasm.[en.wikipedia.org]

Prognosis

  • Multidrug resistant phenotype (positive Pgp expression) is associated with a poor prognosis. Determining the myeloblast-to-erythroblast ratio at diagnosis helps to predict prognosis; a higher ratio is associated with a favorable prognosis.[emedicine.com]
  • Information on prognosis is limited by the rarity of the condition. Prognosis appears to be no different to AML in general, taking into account other risk factors. Acute erythroid leukemia (M6) has a relatively poor prognosis.[en.wikipedia.org]
  • The prognosis of this patient subset is relatively good. The other subset has cytogenetic abnormalities similar to those in myelodysplastic syndromes and a poor prognosis.[ncbi.nlm.nih.gov]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • The etiology of pharyngitis remains obscure in 40% of cases. Most pharyngitis occurs as result of respiratory or contact transmission; few cases are foodborne.[internalmedicinejournal.blogspot.com]
  • Feline leukemia virus has been implicated in the etiology. blast form of erythremic myelosis previously called reticuloendotheliosis, the predominant cell type is more primitive.[medical-dictionary.thefreedictionary.com]
  • All evaluation is necessarily offered with the reservations that must be held regarding a rare disease of unknown etiology.[bloodjournal.org]
  • DIC syndrome can be classified according to etiological, pathogenetic and clinical principles. 13.3.1. Etiological Thrombocytosis Thrombocytosis is divided into primary (tumor, essential) and secondary (reactive and developing after splenectomy).[en.medicine-guidebook.com]

Epidemiology

  • We discuss the differential diagnosis and classification of erythroid neoplasias based upon reproducible hematological criteria to facilitate the gathering and comparison of epidemiological and clinical data on these rare malignancies.[ncbi.nlm.nih.gov]
  • We discuss the differential diagnosis and classification of erythroid neoplasias based upon reproducible hematological criteria to facilitate the gathering and comparison of epidemiological and clinical data on these rare malignancies. 1987 S.[karger.com]
  • 01, 2015) Signs and Symptoms Anemia Circulating erythroblasts Easy bruising or bleeding Fatigue Fever Petechiae Shortness of breath Weakness Diagnostic Exams Immunophenotyping Lumbar puncture Peripheral blood smear Progression and Transformation None Epidemiology[seer.cancer.gov]
  • The PACE (population-adjusted clinical epidemiology) strategy: a new approach to multicentre clinical research. QJM 1997 ; 90 : 147 –51. Office of Population Censuses and Surveys. The 1991 Census on CD-ROM. HMSO, 1995.[jcp.bmj.com]
Sex distribution
Age distribution

Pathophysiology

  • Overlapping SJS and TEN No typical targets, flat atypical targets 10 to 30 TEN No typical targets, flat atypical targets; begins with severe mucosal erosions and progresses to diffuse, generalized detachment of the epidermis Greater than 30 Etiology and Pathophysiology[aafp.org]

Prevention

  • Prevention & Control Active immunization plays a role in prevention with regard to diphtheria and influenza types A and B (Box 9-4). Tonsillectomy is recommended in selected patients.[internalmedicinejournal.blogspot.com]
  • B 29 Continuous acyclovir is recommended to prevent recurrent herpes-associated erythema multiforme.[aafp.org]
  • […] discomfort Mouthwashes to ease discomfort of mouth sores that interferes with eating and drinking Antibiotics for skin infections Corticosteroids to control inflammation Medicines for eye symptoms Good hygiene and staying away from other people may help prevent[medlineplus.gov]
  • You may be given antiviral medication to prevent attacks if you experience them frequently.[nhs.uk]
  • Vitamin B12 level PV may also affect the results of the following tests: ESR Lactate dehydrogenase (LDH) Leukocyte alkaline phosphatase Platelet aggregation test Serum uric acid Treatment The goal of treatment is to reduce the thickness of the blood and prevent[mountsinai.org]

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