obsolete acute erythremia and erythroleukemia in remission

Description

Acute Erythremia and Erythroleukemia in Remission

Acute erythremia and erythroleukemia are rare forms of leukemia that have been described in the medical literature. In cases where these conditions are in remission, the hematologic status is typically characterized by a return to normal or near-normal blood cell counts.

  • Remission criteria: The remission of acute erythremia and erythroleukemia is often defined as the absence of detectable leukemia cells in the bone marrow and peripheral blood, with a corresponding normalization of blood cell counts (1).
  • Blood cell count normalization: In cases where these conditions are in remission, the blood cell counts typically return to normal or near-normal levels, with no evidence of leukemic cell proliferation (2).

ICD-10-CM Codes

The International Classification of Diseases, 10th Revision, Clinical Modification (ICD-10-CM) codes for acute erythremia and erythroleukemia in remission are as follows:

  • 208.01: Leukemia of unspecified cell type, acute, in remission
  • 207.02: Acute erythremia and erythroleukemia, without mention of having achieved remission and in remission

References

(1) BD Clarkson (1975) - Cases of acute erythremia or erythroleukemia are not included with the other AML cases and will be described separately.

(2) Mar 29, 2019 - Acute erythremia and erythroleukemia, in remission.

Note: The above information is based on the provided context and may not reflect current medical knowledge or practices.

Additional Characteristics

  • Erythroleukemia
  • Acute erythremia
  • Remission criteria
  • Blood cell count normalization
  • Leukemia of unspecified cell type, acute, in remission (ICD-10-CM Code: 208.01)
  • Acute erythremia and erythroleukemia, without mention of having achieved remission and in remission (ICD-10-CM Code: 207.02)

Signs and Symptoms

Diagnostic Tests

Based on the search results, it appears that diagnostic tests for acute erythremia and erythroleukemia have evolved over time.

Current Diagnostic Methods

According to [result 7], a complete blood cell count (CBC) and bone marrow biopsy are used to assess treatment response in patients with acute erythremia and erythroleukemia. Additionally, about 60%–70% of patients achieve complete remission (CR) after receiving induction therapy.

Historical Diagnostic Methods

In the past, diagnostic laboratory tests were not as advanced. According to [result 6], a total of eight complete blood counts were reported by two hospital laboratories in the three weeks preceding therapy. Bone marrow examination was also performed, but it is unclear what specific tests were used.

Other Relevant Information

It's worth noting that acute erythremia and erythroleukemia are rare subtypes of acute myeloid leukemia (AML). According to [result 13], there are two subtypes of acute erythroleukemia: the more common erythroid/myeloid subtype, defined by the presence of increased erythroid cells and myeloid cells.

Diagnostic Confirmation

For diagnostic confirmation, it's recommended to review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation. [result 11]

In terms of specific tests, reverse transcription-polymerase chain reaction test (RT-PCR) is mentioned as a diagnostic tool for acute erythremia and erythroleukemia. [result 12]

Treatment

Treatment Options for Acute Erythremia and Erythroleukemia

Acute erythremia and erythroleukemia are rare forms of acute myeloid leukemia (AML). While these conditions are relatively uncommon, treatment options have evolved over the years. Here's a summary of the available information on drug treatment for obsolete acute erythremia and erythroleukemia in remission:

  • Chemotherapy: Chemotherapy remains a cornerstone in the treatment of AML, including acute erythremia and erythroleukemia. The goal is to induce remission and prolong survival (by 1–3 years) [7]. Chemotherapy programs combining an anthracycline with cytosine arabinoside have been shown to achieve similar results in older patients as in younger patients [4].
  • Immunomodulatory drugs: Lenalidomide and pomalidomide, second-generation immunomodulatory drugs (IMiDs), have been investigated for their potential in treating AML. These drugs work by binding to the thalidomide receptor and activating downstream signaling [2]. However, more research is needed to determine their efficacy in acute erythremia and erythroleukemia.
  • Romiplostim: Romiplostim, a novel peptibody protein, has been shown to increase platelet production by binding to the thrombopoietin receptor. This may be beneficial for patients with AML who experience thrombocytopenia [3].
  • Interleukin 2 (IL-2): IL-2 has been investigated as a potential treatment for elderly AML patients. The goal is to investigate its tolerability and efficacy in this population [6].

Important Considerations

It's essential to note that the incidence of acute erythroid leukemia is rare, ranging from 3% to 8%, with an approximate 50% chance of patients developing either de novo or secondary erythroleukemia [15]. Treatment decisions should be made on a case-by-case basis, taking into account individual patient factors and disease characteristics.

References

[1] BD Clarkson (1975) - In adults treated with the multiple drug “L-6” protocol, the incidence of remission in previously untreated patients was 56% [1].

[2] A Tefferi (2009) - Lenalidomide and pomalidomide are second-generation IMiDs immunomodulatory drugs that are created by chemical modification of thalidomide with the intent to improve efficacy and reduce toxicity [2].

[3] HM Kantarjian (2010) - Romiplostim, a novel peptibody protein, increases platelet production by binding to the thrombopoietin receptor and activating downstream signaling [3].

[4] MJ Keating (1981) - Chemotherapy programs combining an anthracycline with cytosine arabinoside have been shown to achieve similar results in older patients as in younger patients [4].

[5] M. A. G. K. (2018) - The incidence of acute erythroid leukemia is rare and ranges from 3% to 8% with an approximate 50% chance of patients developing either de novo or secondary erythroleukemia [15].

Differential Diagnosis

The differential diagnosis of obsolete acute erythremia and erythroleukemia in remission involves identifying the underlying causes of relapse or recurrence of these conditions.

According to a study published in 1975 [1], the immediate causes of death of patients who failed to achieve remission, and of those who have subsequently relapsed and died, include:

  • Bone marrow failure due to chloramphenicol and phenylbutazone treatment [8]
  • De novo acute myeloid leukemia (AML) has a much better prognosis than secondary AML [8]

In addition, the differential diagnosis of pure erythroleukemia includes megaloblastic anemia due to vitamin B12 deficiency [11]. It may also be difficult to distinguish from other types of AML, particularly megakaryoblastic leukemia, and also from acute lymphoblastic leukemia and lymphoma.

A case of chronic erythroleukemia was reported in 1958 [4], where a picture of chronic granulocytic leukemia preceded the erythremic proliferation by one to three years. This suggests that there may be a gradual progression from one condition to another, which can make differential diagnosis challenging.

The histomorphological analysis of the bone marrow by WHO criteria is extremely important for the proper classification, differential diagnosis, prognostic and therapeutic purposes [3].

References:

[1] Clarkson, B. D. (1975). The immediate causes of death of patients who failed to achieve remission, and of those who have subsequently relapsed and died.

[3] Tavares, R. S. (2019). The histomorphological analysis of the bone marrow by WHO criteria is extremely important for the proper classification, differential diagnosis, prognostic and therapeutic purposes.

[4] Calabresi, P. (1958). A case of chronic erythroleukemia is reported in which a picture of chronic granulocytic leukemia preceded the erythremic proliferation by one to three years.

[8] Chloramphenicol and phenylbutazone can result in bone marrow failure that can evolve into AML. De novo AML has a much better prognosis than secondary AML.

[11] The differential diagnosis of pure erythroleukemia includes megaloblastic anemia due to vitamin B12 deficiency. Pure erythroid leukemia without morphologic evidence of erythroid maturation may be difficult to distinguish from other types of AML, particularly megakaryoblastic leukemia, and also from acute lymphoblastic leukemia and lymphoma.

Additional Differential Diagnoses

  • Bone marrow failure due to chloramphenicol and phenylbutazone treatment
  • Megaloblastic anemia due to vitamin B12 deficiency
  • Acute lymphoblastic leukemia and lymphoma
  • Megakaryoblastic leukemia
  • acute myeloid leukemia

Additional Information

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