ICD-10: C93.02

Acute monoblastic/monocytic leukemia, in relapse

Additional Information

Approximate Synonyms

Acute monoblastic/monocytic leukemia (AMoL) is a subtype of acute myeloid leukemia (AML) characterized by the proliferation of monoblasts and promonocytes. The ICD-10 code C93.02 specifically refers to this condition when it is in relapse. Here are some alternative names and related terms associated with this diagnosis:

Alternative Names

  1. Acute Monoblastic Leukemia: This term is often used interchangeably with acute monoblastic/monocytic leukemia, emphasizing the monoblastic component.
  2. Acute Monocytic Leukemia: While this term can refer to a broader category, it is sometimes used to describe cases that may include monoblastic features.
  3. Acute Myeloid Leukemia, Monoblastic Type: This name highlights the classification within the broader category of acute myeloid leukemia.
  1. Relapsed Acute Monoblastic Leukemia: This term specifically indicates that the leukemia has returned after a period of remission.
  2. Acute Leukemia: A general term that encompasses all types of acute leukemia, including both lymphoblastic and myeloid subtypes.
  3. Monocytic Leukemia: A broader term that may refer to any leukemia involving monocytic cells, including both acute and chronic forms.
  4. Myeloid Neoplasm: This term includes various disorders of the myeloid lineage, which encompasses acute myeloid leukemia and its subtypes.
  5. Leukemia, Monoblastic Type: A descriptive term that may be used in clinical settings to specify the type of leukemia.

Clinical Context

Acute monoblastic/monocytic leukemia is part of the World Health Organization (WHO) classification of hematological malignancies and is characterized by specific clinical and laboratory features. The relapse of this condition indicates a return of the disease after treatment, which can significantly impact management strategies and prognosis.

Understanding these alternative names and related terms is crucial for healthcare professionals involved in the diagnosis, treatment, and coding of this condition, ensuring accurate communication and documentation in clinical practice.

Diagnostic Criteria

Acute monoblastic/monocytic leukemia (AMoL) is a subtype of acute myeloid leukemia (AML) characterized by the proliferation of monoblasts and monocytic cells. The diagnosis of AMoL, particularly in the context of relapse, involves a combination of clinical, laboratory, and cytogenetic criteria. Below is a detailed overview of the criteria used for diagnosing ICD-10 code C93.02, which specifically refers to acute monoblastic/monocytic leukemia in relapse.

Clinical Presentation

  1. Symptoms: Patients may present with symptoms typical of leukemia, including:
    - Fatigue and weakness
    - Fever and infections due to neutropenia
    - Bleeding tendencies (e.g., petechiae, bruising)
    - Bone pain or discomfort

  2. Physical Examination: Findings may include:
    - Splenomegaly (enlarged spleen)
    - Hepatomegaly (enlarged liver)
    - Lymphadenopathy (swollen lymph nodes)

Laboratory Findings

  1. Complete Blood Count (CBC):
    - Anemia (low hemoglobin levels)
    - Thrombocytopenia (low platelet count)
    - Leukocytosis or leukopenia (elevated or decreased white blood cell count)

  2. Bone Marrow Biopsy:
    - A definitive diagnosis is made through a bone marrow biopsy, which typically shows:

    • Greater than 20% blasts in the bone marrow
    • Presence of monoblasts or promonocytes, which are indicative of monoblastic/monocytic lineage.
  3. Cytochemical Staining:
    - Specific stains (e.g., myeloperoxidase, nonspecific esterase) can help differentiate between myeloid and monocytic lineages.

Cytogenetic and Molecular Studies

  1. Cytogenetic Analysis:
    - Karyotyping may reveal chromosomal abnormalities associated with acute myeloid leukemia, such as translocations or deletions that can influence prognosis and treatment.

  2. Molecular Testing:
    - Testing for mutations in genes such as FLT3, NPM1, and others can provide additional diagnostic and prognostic information.

Diagnostic Criteria for Relapse

  1. Definition of Relapse:
    - Relapse is defined as the reappearance of leukemic blasts in the peripheral blood or bone marrow after a period of remission. This typically involves:

    • At least 5% blasts in the bone marrow or peripheral blood.
    • Clinical symptoms of leukemia re-emerging.
  2. Monitoring:
    - Regular monitoring of blood counts and bone marrow status is essential for early detection of relapse.

Conclusion

The diagnosis of acute monoblastic/monocytic leukemia in relapse (ICD-10 code C93.02) relies on a combination of clinical symptoms, laboratory findings, and cytogenetic analysis. A thorough evaluation by a hematologist is crucial for accurate diagnosis and appropriate management. If you have further questions or need more specific information regarding treatment options or prognosis, feel free to ask!

Treatment Guidelines

Acute monoblastic/monocytic leukemia (AMML), classified under ICD-10 code C93.02, is a subtype of acute myeloid leukemia (AML) characterized by the proliferation of monoblasts and promonocytes. When this condition relapses, treatment approaches typically involve a combination of chemotherapy, targeted therapies, and supportive care. Below is a detailed overview of the standard treatment strategies for managing relapsed AMML.

Treatment Approaches for Relapsed Acute Monoblastic/Monocytic Leukemia

1. Chemotherapy

Chemotherapy remains the cornerstone of treatment for relapsed AMML. The primary goal is to achieve remission by eliminating leukemic cells. Common regimens include:

  • Re-induction Therapy: This often involves using a similar regimen to the initial treatment, which may include cytarabine combined with an anthracycline (e.g., daunorubicin or idarubicin). The choice of drugs may depend on the patient's previous response and tolerance to treatment[1].

  • High-Dose Cytarabine: In some cases, high-dose cytarabine may be used, especially if the patient had a good response to this agent during initial treatment. This approach aims to intensify the treatment and improve outcomes[2].

2. Targeted Therapy

Recent advancements in targeted therapies have provided new options for patients with relapsed AMML. These therapies focus on specific genetic mutations or abnormalities present in the leukemic cells:

  • FLT3 Inhibitors: For patients with FLT3 mutations, drugs like midostaurin or gilteritinib may be utilized to target these mutations specifically, improving the chances of achieving remission[3].

  • IDH Inhibitors: If the patient has mutations in the isocitrate dehydrogenase (IDH) genes, therapies such as ivosidenib or enasidenib can be effective in targeting these pathways[4].

3. Stem Cell Transplantation

For patients who achieve remission after chemotherapy, hematopoietic stem cell transplantation (HSCT) may be considered, especially in cases of high-risk disease or those with a second relapse. This procedure can provide a potential cure by replacing the diseased bone marrow with healthy stem cells from a donor[5].

  • Allogeneic Transplantation: This is often preferred for relapsed AMML, as it can help prevent further relapses by utilizing the donor's immune system to attack any remaining leukemic cells[6].

4. Supportive Care

Supportive care is crucial in managing the side effects of treatment and improving the quality of life for patients. This includes:

  • Transfusions: Patients may require red blood cell or platelet transfusions to manage anemia and thrombocytopenia, which are common during treatment[7].

  • Infection Prophylaxis: Due to the immunocompromised state of patients undergoing chemotherapy, prophylactic antibiotics and antifungals are often administered to prevent infections[8].

  • Nutritional Support: Maintaining adequate nutrition is essential, as patients may experience decreased appetite and gastrointestinal side effects from treatment[9].

5. Clinical Trials

Participation in clinical trials may also be an option for patients with relapsed AMML. These trials often explore new therapies or combinations of existing treatments, providing access to cutting-edge care that may improve outcomes[10].

Conclusion

The management of relapsed acute monoblastic/monocytic leukemia involves a multifaceted approach that includes chemotherapy, targeted therapies, stem cell transplantation, and supportive care. Each treatment plan should be tailored to the individual patient's needs, considering factors such as previous treatment responses, genetic mutations, and overall health status. Ongoing research and clinical trials continue to enhance the understanding and treatment of this challenging condition, offering hope for improved outcomes in the future.

Description

Acute monoblastic/monocytic leukemia (AMML), classified under ICD-10 code C93.02, is a subtype of acute myeloid leukemia (AML) characterized by the proliferation of monoblasts and promonocytes in the bone marrow and peripheral blood. This condition is particularly aggressive and is associated with a range of clinical manifestations and complications.

Clinical Description

Definition and Classification

Acute monoblastic/monocytic leukemia is defined as a hematological malignancy that arises from the myeloid lineage, specifically affecting monocytes. It is classified under the broader category of acute myeloid leukemia, which encompasses various subtypes based on the specific lineage of the leukemic cells. C93.02 specifically denotes cases where the disease is in relapse, indicating a return of the disease after a period of remission[1][2].

Symptoms

Patients with AMML may present with a variety of symptoms, which can include:
- Fatigue and Weakness: Due to anemia resulting from bone marrow infiltration.
- Fever and Infections: Increased susceptibility to infections due to leukopenia (low white blood cell count).
- Bleeding and Bruising: Caused by thrombocytopenia (low platelet count), leading to easy bruising and prolonged bleeding from minor injuries.
- Bone Pain: Resulting from the expansion of leukemic cells in the bone marrow.
- Lymphadenopathy and Splenomegaly: Enlargement of lymph nodes and spleen due to leukemic infiltration[3][4].

Diagnosis

Diagnosis of AMML typically involves:
- Blood Tests: Complete blood count (CBC) showing anemia, leukopenia, or leukocytosis.
- Bone Marrow Biopsy: Essential for confirming the diagnosis, revealing a high percentage of monoblasts.
- Cytogenetic and Molecular Studies: To identify specific genetic abnormalities associated with the disease, which can influence prognosis and treatment strategies[5][6].

Treatment and Management

The management of acute monoblastic/monocytic leukemia, particularly in relapse, often involves:
- Chemotherapy: Intensive chemotherapy regimens aimed at achieving remission.
- Targeted Therapy: Depending on the genetic profile of the leukemia, targeted therapies may be employed.
- Stem Cell Transplantation: Considered for eligible patients, especially in cases of relapse, to provide a potential cure by replacing the diseased bone marrow with healthy stem cells[7][8].

Prognosis

The prognosis for patients with AMML can vary significantly based on several factors, including the patient's age, overall health, the presence of specific genetic mutations, and the response to initial treatment. Relapsed cases generally have a poorer prognosis compared to newly diagnosed cases, necessitating aggressive treatment approaches[9][10].

Conclusion

ICD-10 code C93.02 captures the complexity of acute monoblastic/monocytic leukemia in relapse, highlighting the need for comprehensive clinical evaluation and tailored treatment strategies. Understanding the clinical features, diagnostic criteria, and management options is crucial for healthcare providers in delivering effective care for patients facing this challenging condition.

For further information or specific case management strategies, consulting hematology-oncology specialists is recommended.

Clinical Information

Acute monoblastic/monocytic leukemia (AMML), classified under ICD-10 code C93.02, is a subtype of acute myeloid leukemia (AML) characterized by the proliferation of monoblasts and promonocytes. This condition can present with a variety of clinical features, signs, and symptoms, particularly when the disease is in relapse. Understanding these aspects is crucial for timely diagnosis and management.

Clinical Presentation

Signs and Symptoms

  1. Hematological Manifestations:
    - Anemia: Patients often present with fatigue, pallor, and weakness due to decreased red blood cell production.
    - Thrombocytopenia: Low platelet counts can lead to easy bruising, petechiae, and increased bleeding tendencies.
    - Leukopenia or Leukocytosis: Depending on the stage of the disease, patients may exhibit low white blood cell counts or elevated counts, which can lead to increased susceptibility to infections.

  2. Organomegaly:
    - Splenomegaly: Enlargement of the spleen is common and can cause discomfort or a feeling of fullness.
    - Hepatomegaly: Liver enlargement may also occur, contributing to abdominal pain or discomfort.

  3. Extramedullary Involvement:
    - Leukemia Cutis: Skin lesions may appear as a result of leukemic infiltration, presenting as nodules or plaques.
    - Gingival Hyperplasia: Swelling and bleeding of the gums can occur due to infiltration of leukemic cells.

  4. Neurological Symptoms:
    - In cases of central nervous system involvement, patients may experience headaches, seizures, or altered mental status.

  5. Fever and Infection:
    - Patients may present with fever due to infections, which are common due to immunosuppression from the disease and treatment.

Patient Characteristics

  • Age: Acute monoblastic/monocytic leukemia can occur at any age but is more prevalent in adults, particularly those over 60 years old.
  • Gender: There is a slight male predominance in the incidence of AML, including AMML.
  • Previous History: A significant number of patients may have a history of prior hematological disorders or exposure to risk factors such as chemotherapy or radiation therapy, which can predispose them to relapse.
  • Genetic Factors: Certain genetic mutations and chromosomal abnormalities, such as those involving the FLT3 or NPM1 genes, may be associated with a higher risk of relapse and poor prognosis.

Conclusion

Acute monoblastic/monocytic leukemia in relapse presents with a complex array of clinical features, including hematological abnormalities, organomegaly, and potential extramedullary involvement. Recognizing these signs and symptoms is essential for healthcare providers to facilitate early diagnosis and appropriate management. Understanding patient characteristics, such as age, gender, and previous medical history, can also aid in tailoring treatment strategies and improving outcomes for individuals affected by this aggressive form of leukemia.

Related Information

Approximate Synonyms

  • Acute Monoblastic Leukemia
  • Acute Monocytic Leukemia
  • Monoblastic Type AML
  • Relapsed Acute Monoblastic Leukemia
  • Leukemia, Monoblastic Type

Diagnostic Criteria

  • Proliferation of monoblasts and monocytic cells
  • Greater than 20% blasts in bone marrow
  • Monocytic lineage confirmed by cytochemical staining
  • At least 5% blasts in bone marrow or peripheral blood
  • Clinical symptoms of leukemia re-emerging

Treatment Guidelines

  • Chemotherapy remains cornerstone of treatment
  • Re-induction therapy with cytarabine and anthracycline
  • High-dose cytarabine may be used for intensive treatment
  • FLT3 inhibitors target FLT3 mutations specifically
  • IDH inhibitors target IDH gene mutations
  • Stem cell transplantation provides potential cure
  • Allogeneic transplantation preferred for relapsed AMML
  • Supportive care manages treatment side effects
  • Transfusions manage anemia and thrombocytopenia
  • Infection prophylaxis prevents infections during treatment
  • Nutritional support maintains adequate nutrition

Description

  • Acute myeloid leukemia subtype
  • Monoblasts and promonocytes proliferation
  • Bone marrow and peripheral blood involvement
  • Aggressive clinical course
  • Fatigue and weakness common symptoms
  • Fever and infections frequent complications
  • Bleeding and bruising due to thrombocytopenia
  • Bone pain from leukemic cell expansion
  • Lymphadenopathy and splenomegaly present

Clinical Information

  • Anemia presents with fatigue and weakness
  • Thrombocytopenia causes easy bruising and bleeding
  • Leukopenia or leukocytosis increases infection risk
  • Splenomegaly is common and painful
  • Hepatomegaly contributes to abdominal pain
  • Leukemia Cutis appears as skin nodules or plaques
  • Gingival Hyperplasia causes gum swelling and bleeding
  • Central Nervous System involvement causes headaches and seizures
  • Fever and infections are common due to immunosuppression
  • Male predominance in incidence of AMML
  • Adults over 60 years old have higher risk
  • Prior hematological disorders increase relapse risk

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.