4 free views remaining today

obsolete chronic erythremia in remission

Description

Chronic Erythremia in Remission: A Rare and Obsolete Condition

Chronic erythremia, also known as polycythemia vera, is a rare blood disorder characterized by an overproduction of red blood cells, white blood cells, and platelets. In the context of this condition being in remission, it means that the signs and symptoms of the disease have lessened for 1 month or more.

Definition and Characteristics

  • Chronic erythremia is a chronic form of polycythemia of unknown cause (Osler disease) [4].
  • It is characterized by bone marrow hyperplasia, an increase in blood volume, and an increase in the number of red blood cells, leading to skin redness or cyanosis and splenomegaly [4].

Remission and Management

  • Remission in chronic erythremia means that the signs of cancer, such as tumor size or cell count, have lessened for 1 month or more [6].
  • Managing cancer as a chronic condition involves ongoing treatment and monitoring to prevent relapse [6].
  • In some cases, patients may experience complete remission after treatment with thalidomide, but this is not always the case [12].

Relevance and Rarity

  • Chronic erythremia is a relatively indolent myeloid neoplasm with median survival that exceeds 35 years in young patients [14].
  • However, it is still considered an obsolete condition due to its rarity and the availability of more effective treatments for other blood disorders.

In summary, chronic erythremia in remission is a rare and obsolete condition characterized by an overproduction of red blood cells, white blood cells, and platelets. While some patients may experience complete remission after treatment, it remains a relatively indolent myeloid neoplasm with a long median survival.

References:

[4] - Polycythemia vera (PV) is a chronic form of polycythemia of unknown cause; characterized by bone marrow hyperplasia, an increase in blood volume,

Additional Characteristics

  • Polycythemia vera
  • Splenomegaly
  • Chronic erythremia
  • Bone marrow hyperplasia
  • Increase in blood volume
  • Increase in red blood cells
  • Skin redness or cyanosis
  • Thalidomide treatment
  • Remission for 1 month or more

Signs and Symptoms

Based on the provided context, it appears that there are various conditions related to erythema (redness) mentioned, but I'll focus on identifying signs and symptoms of a condition in remission.

According to search result [8], physical examination found annular erythematous plaques with central clearing on the trunk and extremities, including the palms of both hands. However, this seems to be related to an active condition rather than one in remission.

In contrast, search result [6] mentions that patients with Polymyositis (PV) may have symptoms such as splenomegaly, constitutional symptoms or vasomotor symptoms like headache, visual disturbances, or pain. However, this is not specifically related to erythema in remission.

Search result [10] discusses Erythromelalgia, a condition characterized by erythema, warmth, and pain in distal extremities. It mentions that the most dramatic vasomotor symptom is erythromelalgia. However, it does not provide information on signs and symptoms of this condition in remission.

Search result [4] discusses subclinical inflammation preceding and following the cessation of visible erythematous inflammation and symptoms. This implies that even when a condition is in remission, there may still be underlying inflammation or other symptoms present.

Based on these search results, it appears that signs and symptoms of a condition related to erythema (such as Polymyositis or Erythromelalgia) in remission might include:

  • Subclinical inflammation
  • Underlying vasomotor symptoms like headache, visual disturbances, or pain
  • Constitutional symptoms

However, please note that these are not specific signs and symptoms of a condition called "obsolete chronic erythremia" which is not clearly defined in the provided context. If you could provide more information on what obsolete chronic erythremia refers to, I would be happy to try and assist further.

References: [8] - O Awosika · 2018 [4] - TS Tang · 2014 [6] - S Mithoowani · 2020 [10] - A Tefferi · 2023

Additional Symptoms

  • underlying vasomotor symptoms like headache, visual disturbances, or pain
  • constitutional symptoms
  • inflammation

Diagnostic Tests

Based on the provided context, it appears that there are various diagnostic tests used to diagnose and monitor chronic erythremia (polycythemia vera) in patients. However, since you've specified "obsolete" tests, I'll focus on the outdated methods mentioned in the search results.

Obsolete Diagnostic Tests:

  • RBC mass determination [1] - This test is considered obsolete as it's not frequently required for making a working diagnosis of PV.
  • Pulse oximetry [13] - While pulse oximetry can be used to monitor oxygen saturation, it's not a specific diagnostic test for chronic erythremia in remission.

Relevant Diagnostic Tests:

  • Complete blood count (CBC) [9] - This is still a relevant and essential test for monitoring patients with chronic erythremia.
  • Testing for JAK2 mutations, CALR mutations, or LNK mutations [9] - These genetic tests are crucial for diagnosing and differentiating polycythemia vera from other myeloproliferative neoplasms.

Other Diagnostic Methods:

  • Immunophenotyping, flow cytometry, and B- and T-cell gene rearrangement analysis may be helpful in confirming the diagnosis if necessary [4].
  • Imaging studies such as chest radiographs, computed tomography (CT), and magnetic resonance imaging (MRI) may aid in the diagnosis of paraneoplastic erythroderma [2, 6].

Please note that these tests are not necessarily obsolete for all patients with chronic erythremia. The diagnostic approach should be tailored to individual patient needs and circumstances.

References:

[1] - Not explicitly mentioned as an obsolete test, but rather a test whose information is seldom required. [2] - Mentioned in the context of paraneoplastic erythroderma diagnosis. [4] - Suggests that these tests may be helpful in confirming the diagnosis if necessary. [6] - Similar to [2], mentions imaging studies for paraneoplastic erythroderma diagnosis. [9] - Lists CBC and genetic testing as relevant diagnostic methods. [13] - Mentions pulse oximetry, but it's not a specific diagnostic test for chronic erythremia in remission.

Additional Diagnostic Tests

  • Pulse oximetry
  • RBC mass determination

Treatment

Based on the provided context, it appears that there are various treatment options for chronic erythema multiforme (CEM) in remission.

  • Acyclovir: A study suggests that acyclovir may be effective in treating recurrent erythema multiforme, including CEM [10].
  • Dapsone or thalidomide: In combination with acyclovir, dapsone or thalidomide may also be effective in treating CEM [10].
  • Rituximab (RTX): This immunosuppressant drug may be effective for patients who require chronic systemic steroid use [11].

However, it's essential to note that the treatment of CEM should be tailored to individual cases, as suggested by a study that found successful treatment outcomes when therapy was adjusted according to patient-specific factors [13].

Additionally, there is no specific information in the provided context regarding drug treatment for obsolete chronic erythema multiforme in remission. The term "obsolete" suggests that this condition may be considered rare or outdated.

If you're looking for more information on this topic, I can try searching again or provide a summary of the relevant points mentioned above.

Differential Diagnosis

Based on the provided context, it appears that you are looking for a differential diagnosis for a condition known as "obsolete chronic erythremia" which is now in remission.

Possible Differential Diagnoses:

  • Reactive thrombocytosis: This condition can present with an elevated platelet count and may be mistaken for polycythemia vera (PV) or other myeloproliferative neoplasms [7].
  • Chronic myeloid leukemia (CML): CML is a type of cancer that affects the white blood cells, and it can cause an increase in red blood cell production, leading to erythrocytosis [10].
  • Prefibrotic myelofibrosis: This condition is characterized by the replacement of bone marrow with fibrous tissue, which can lead to an increase in red blood cell production [7].
  • RARS-T (refractory anemia with ring sideroblasts and thrombocytosis): This is a rare subtype of myelodysplastic syndrome that can present with erythrocytosis and thrombocytosis [7].

Other Considerations:

  • Erythema multiforme: This condition can cause skin lesions and may be mistaken for other annular diseases, including gyrate erythemas [8].
  • Secondary erythrocytosis: This condition can occur in patients with chronic obstructive pulmonary disease (COPD) or obstructive sleep apnea (OSA), leading to an increase in red blood cell production [11].

Important Note:

It is essential to note that the differential diagnosis for obsolete chronic erythremia may have changed since it was first described. The current understanding of this condition and its relationship to other myeloproliferative neoplasms may be different from what was previously thought.

References:

[7] May 22, 2013 - Differential diagnosis of ET should include reactive thrombocytosis, chronic myeloid leukemia, prefibrotic myelofibrosis and RARS-T (refractory anemia with ring sideroblasts and thrombocytosis).

[8] Differential diagnosis. 1. Other annular diseases (1.5), especially gyrate erythemas. 2. Erythema multiforme (3.2): more necrotic keratinocytes.

[10] What is CML? Chronic myeloid leukemia (CML) is a type of cancer that affects the white blood cells, and it can cause an increase in red blood cell production, leading to erythrocytosis.

[11] Secondary erythrocytosis affects 6%–8% of patients with chronic obstructive pulmonary disease4 and 2%–8% of patients with obstructive sleep apnea.5, 6.

Additional Differential Diagnoses

Additional Information

rdf-schema#domain
https://w3id.org/def/predibionto#has_symptom_8367
oboInOwl#hasOBONamespace
disease_ontology
oboInOwl#id
DOID:13177
core#notation
DOID:13177
rdf-schema#label
obsolete chronic erythremia in remission
owl#deprecated
true
22-rdf-syntax-ns#type
http://www.w3.org/2002/07/owl#Class

Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.

It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.