ICD-10: C91.00
Acute lymphoblastic leukemia not having achieved remission
Clinical Information
Inclusion Terms
- Acute lymphoblastic leukemia NOS
- Acute lymphoblastic leukemia with failed remission
Additional Information
Clinical Information
Acute Lymphoblastic Leukemia (ALL) is a type of cancer that affects the blood and bone marrow, characterized by the overproduction of immature lymphocytes. The ICD-10 code C91.00 specifically refers to cases of Acute Lymphoblastic Leukemia that have not achieved remission. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for effective diagnosis and management.
Clinical Presentation
Signs and Symptoms
Patients with Acute Lymphoblastic Leukemia typically present with a range of symptoms that can vary in severity. Common signs and symptoms include:
- Fatigue and Weakness: Due to anemia, patients often experience significant fatigue and a general sense of weakness, which can impact daily activities[1].
- Fever and Infections: The disease can lead to neutropenia (low white blood cell count), making patients more susceptible to infections. Fever may be a common presenting symptom[1].
- Bleeding and Bruising: Patients may notice easy bruising, frequent nosebleeds, or bleeding gums, which are indicative of thrombocytopenia (low platelet count)[1].
- Bone Pain: Patients often report pain in the bones or joints, which can be attributed to the infiltration of leukemic cells in the bone marrow[1].
- Swollen Lymph Nodes: Lymphadenopathy (swelling of lymph nodes) is common, particularly in the neck, armpits, and groin[1].
- Splenomegaly and Hepatomegaly: Enlargement of the spleen and liver may occur, leading to abdominal discomfort or fullness[1].
Patient Characteristics
The characteristics of patients diagnosed with ALL not in remission can vary widely, but several key factors are often observed:
- Age: While ALL is more common in children, adults can also be affected. The prognosis and treatment response may differ significantly between pediatric and adult populations[1].
- Gender: There is a slight male predominance in the incidence of ALL, although the reasons for this are not fully understood[1].
- Cytogenetic Abnormalities: Certain genetic mutations and chromosomal abnormalities, such as Philadelphia chromosome positivity, can influence the disease's behavior and response to treatment[1].
- Previous Treatment History: Patients who have undergone prior treatments for leukemia or other malignancies may have different clinical presentations and responses to therapy[1].
Conclusion
Acute Lymphoblastic Leukemia not having achieved remission (ICD-10 code C91.00) presents with a variety of clinical signs and symptoms, including fatigue, fever, bleeding tendencies, and bone pain. Patient characteristics such as age, gender, and genetic factors play a significant role in the disease's progression and treatment outcomes. Understanding these aspects is essential for healthcare providers to tailor appropriate management strategies and improve patient care. Further research and clinical observation are necessary to enhance treatment protocols and outcomes for this challenging condition.
Approximate Synonyms
Acute lymphoblastic leukemia (ALL) is a type of cancer that affects the blood and bone marrow, characterized by the overproduction of immature white blood cells. The ICD-10 code C91.00 specifically refers to "Acute lymphoblastic leukemia not having achieved remission." Here, we will explore alternative names and related terms associated with this condition.
Alternative Names for Acute Lymphoblastic Leukemia
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Acute Lymphocytic Leukemia (ALL): This term is often used interchangeably with acute lymphoblastic leukemia, although it may refer more broadly to the same disease.
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Lymphoblastic Leukemia: A simplified term that emphasizes the lymphoblastic nature of the leukemia.
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Acute Lymphoblastic Lymphoma: In some cases, ALL can present as a lymphoma, particularly in the mediastinal region, leading to this alternative designation.
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Precursor B-cell Lymphoblastic Leukemia: This term specifies the type of lymphoblasts involved, particularly in cases where the leukemia originates from B-cell precursors.
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Precursor T-cell Lymphoblastic Leukemia: Similar to the above, this term is used when the leukemia arises from T-cell precursors.
Related Terms
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Minimal Residual Disease (MRD): This term refers to the small number of cancer cells that may remain in a patient after treatment, which is significant in assessing the risk of relapse in ALL.
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Remission: A state in which the signs and symptoms of leukemia are reduced or absent. The term "not having achieved remission" indicates that the disease is still active.
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Cytogenetic Abnormalities: Refers to chromosomal changes that can be associated with ALL, which may influence prognosis and treatment strategies.
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Bone Marrow Biopsy: A diagnostic procedure often used to confirm the presence of ALL and assess the extent of the disease.
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Chemotherapy: The primary treatment modality for ALL, which may include various drug regimens aimed at inducing remission.
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Targeted Therapy: Treatments such as Blinatumomab (Blincyto) that specifically target certain characteristics of the leukemia cells, often used in cases of relapsed or refractory ALL.
Conclusion
Understanding the alternative names and related terms for ICD-10 code C91.00 is crucial for healthcare professionals involved in the diagnosis and treatment of acute lymphoblastic leukemia. These terms not only facilitate clearer communication among medical practitioners but also enhance patient education regarding their condition and treatment options. If you have further questions or need more specific information, feel free to ask!
Diagnostic Criteria
Acute lymphoblastic leukemia (ALL) is a type of cancer that affects the blood and bone marrow, characterized by the overproduction of immature lymphocytes. The diagnosis of ALL, particularly when it has not achieved remission, involves a combination of clinical evaluation, laboratory tests, and specific criteria outlined in the ICD-10 coding system.
Diagnostic Criteria for Acute Lymphoblastic Leukemia (ICD-10 Code C91.00)
1. Clinical Presentation
Patients with ALL typically present with a range of symptoms that may include:
- Fatigue and Weakness: Due to anemia from low red blood cell counts.
- Frequent Infections: Resulting from neutropenia (low white blood cell counts).
- Easy Bruising or Bleeding: Caused by thrombocytopenia (low platelet counts).
- Bone Pain: Often due to leukemic infiltration of the bone marrow.
- Lymphadenopathy and Splenomegaly: Enlargement of lymph nodes and spleen due to leukemic cell accumulation.
2. Laboratory Tests
Diagnosis is confirmed through various laboratory tests, including:
- Complete Blood Count (CBC): This test typically shows elevated white blood cell counts with a predominance of lymphoblasts, low red blood cells, and low platelets.
- Bone Marrow Biopsy: A definitive test where a sample of bone marrow is examined for the presence of lymphoblasts. A diagnosis of ALL is made if more than 20% of the cells in the bone marrow are lymphoblasts.
- Cytogenetic Analysis: This involves examining the chromosomes of the leukemic cells to identify specific genetic abnormalities associated with ALL, such as the Philadelphia chromosome.
3. Immunophenotyping
Flow cytometry is used to determine the specific type of lymphoblasts present. This helps classify ALL into subtypes (e.g., B-cell or T-cell ALL) and is crucial for treatment planning.
4. Minimal Residual Disease (MRD) Assessment
For cases where ALL has not achieved remission, assessing minimal residual disease is critical. MRD testing can detect small numbers of leukemic cells that may remain after treatment, indicating the likelihood of relapse. This is often done using sensitive techniques such as polymerase chain reaction (PCR) or flow cytometry.
5. ICD-10 Coding
The ICD-10 code for acute lymphoblastic leukemia not having achieved remission is C91.00. This code is specifically used when the disease is active and has not responded to initial treatment, indicating the need for further therapeutic interventions.
Conclusion
The diagnosis of acute lymphoblastic leukemia, particularly in cases where remission has not been achieved, relies on a comprehensive evaluation that includes clinical symptoms, laboratory findings, and advanced diagnostic techniques. Accurate coding with ICD-10 C91.00 is essential for appropriate treatment planning and healthcare management. Understanding these criteria is vital for healthcare professionals involved in the diagnosis and treatment of patients with ALL.
Treatment Guidelines
Acute Lymphoblastic Leukemia (ALL), classified under ICD-10 code C91.00, is a type of cancer that affects the blood and bone marrow. When a patient with ALL has not achieved remission, it indicates that the disease is still active and requires immediate and effective treatment strategies. Below, we explore the standard treatment approaches for this condition.
Overview of Acute Lymphoblastic Leukemia
Acute Lymphoblastic Leukemia is characterized by the rapid proliferation of immature lymphocytes, known as lymphoblasts, in the bone marrow and blood. This condition can affect both children and adults, though treatment protocols may vary based on age, overall health, and specific disease characteristics.
Standard Treatment Approaches
1. Induction Therapy
The first step in treating ALL that has not achieved remission is typically induction therapy. This phase aims to eliminate as many leukemic cells as possible and induce remission. Induction therapy usually involves:
- Chemotherapy: A combination of drugs is administered, often including agents such as vincristine, prednisone, and anthracyclines (e.g., daunorubicin or doxorubicin). The specific regimen may vary based on the patient's age and genetic factors of the leukemia[1].
- Supportive Care: Patients may require transfusions of red blood cells and platelets, as well as antibiotics to prevent infections due to neutropenia (low white blood cell count) caused by chemotherapy[1].
2. Consolidation Therapy
Once remission is achieved, consolidation therapy follows to eliminate any remaining leukemic cells. This phase may include:
- Continuation of Chemotherapy: Higher doses or different combinations of chemotherapy drugs are often used to target residual disease.
- Central Nervous System (CNS) Prophylaxis: Given the risk of leukemia spreading to the CNS, intrathecal chemotherapy (directly into the spinal fluid) may be administered to prevent or treat CNS involvement[2].
3. Maintenance Therapy
After consolidation, maintenance therapy is typically employed to sustain remission. This phase usually involves:
- Oral Chemotherapy: Medications such as mercaptopurine and methotrexate are commonly used in lower doses over an extended period, often lasting two to three years[2].
- Regular Monitoring: Frequent blood tests and clinical evaluations are necessary to monitor for signs of relapse and manage any side effects of treatment.
4. Targeted Therapy
For patients with specific genetic mutations or characteristics, targeted therapies may be considered. For example:
- Tyrosine Kinase Inhibitors (TKIs): In cases where the leukemia is associated with the Philadelphia chromosome (BCR-ABL fusion), drugs like imatinib or dasatinib may be used in conjunction with chemotherapy[3].
- Monoclonal Antibodies: Agents such as blinatumomab, which targets CD19 on B-cell leukemias, can be effective in relapsed or refractory cases[3].
5. Stem Cell Transplantation
For patients who do not respond to initial treatments or have a high risk of relapse, hematopoietic stem cell transplantation (HSCT) may be considered. This procedure involves:
- High-Dose Chemotherapy: Administered to eradicate the leukemic cells before transplanting healthy stem cells from a donor or the patient (autologous transplant)[4].
- Post-Transplant Care: Close monitoring for complications such as graft-versus-host disease (GVHD) and infections is crucial following the transplant[4].
Conclusion
The treatment of Acute Lymphoblastic Leukemia that has not achieved remission is complex and requires a multi-faceted approach involving induction, consolidation, and maintenance therapies, along with potential targeted therapies and stem cell transplantation. Each treatment plan should be tailored to the individual patient, considering their specific disease characteristics and overall health. Continuous monitoring and supportive care are essential to manage side effects and improve outcomes. For patients and families, engaging with a specialized oncology team is critical to navigating the challenges of this aggressive disease.
References
- Agency for Health Technology Assessment and Tariff System.
- Medical Drug Clinical Criteria.
- Billing and Coding: Biomarkers for Oncology (A52986).
- HCT for Acute Lymphoblastic Leukemia.
Description
Acute lymphoblastic leukemia (ALL) is a type of cancer that affects the blood and bone marrow, characterized by the overproduction of immature lymphocytes, a type of white blood cell. The ICD-10 code C91.00 specifically refers to cases of acute lymphoblastic leukemia that have not achieved remission, indicating a persistent disease state despite treatment efforts.
Clinical Description of Acute Lymphoblastic Leukemia (ALL)
Overview
Acute lymphoblastic leukemia is primarily seen in children but can also occur in adults. It is classified as a hematological malignancy and is characterized by the rapid proliferation of lymphoblasts, which are immature lymphocytes. The disease can lead to a variety of symptoms due to the infiltration of leukemic cells into the bone marrow and other organs, disrupting normal blood cell production.
Symptoms
Patients with ALL may present with a range of symptoms, including:
- Fatigue and weakness: Due to anemia from reduced red blood cell production.
- Frequent infections: Resulting from neutropenia (low white blood cell count).
- Easy bruising or bleeding: Caused by thrombocytopenia (low platelet count).
- Bone pain: Often due to the expansion of leukemic cells in the bone marrow.
- Swollen lymph nodes: Indicative of lymphatic involvement.
- Fever and night sweats: Common systemic symptoms associated with malignancy.
Diagnosis
Diagnosis of ALL typically involves:
- Blood tests: To assess blood cell counts and identify abnormal lymphoblasts.
- Bone marrow biopsy: To confirm the presence of leukemic cells and determine the extent of the disease.
- Cytogenetic analysis: To identify specific genetic abnormalities that may influence prognosis and treatment.
ICD-10 Code C91.00: Specifics
Definition
The ICD-10 code C91.00 is designated for acute lymphoblastic leukemia that has not achieved remission. This classification is crucial for treatment planning and billing purposes, as it indicates a more severe disease state that requires ongoing management.
Treatment Considerations
For patients with ALL who have not achieved remission, treatment options may include:
- Intensified chemotherapy regimens: To attempt to induce remission.
- Targeted therapies: Such as tyrosine kinase inhibitors for specific genetic mutations.
- Stem cell transplantation: Considered for eligible patients, especially if there is a suitable donor.
- Clinical trials: Exploring new therapies that may be effective in refractory cases.
Prognosis
The prognosis for patients with ALL who have not achieved remission is generally poor compared to those who respond to initial treatment. Factors influencing outcomes include:
- Age at diagnosis: Younger patients often have better outcomes.
- Genetic factors: Certain chromosomal abnormalities can indicate a more favorable or unfavorable prognosis.
- Response to initial therapy: Early response to treatment is a strong predictor of long-term outcomes.
Conclusion
ICD-10 code C91.00 is critical for identifying and managing cases of acute lymphoblastic leukemia that have not achieved remission. Understanding the clinical implications, treatment options, and prognosis associated with this diagnosis is essential for healthcare providers to deliver effective care and support to affected patients. Continuous monitoring and adjustment of treatment strategies are vital in managing this challenging condition.
Related Information
Clinical Information
- Fatigue due to anemia common
- Fever indicative of infections
- Bleeding tendencies due to thrombocytopenia
- Bone pain from leukemic cell infiltration
- Swollen lymph nodes a common symptom
- Splenomegaly and hepatomegaly possible
- Male predominance in incidence observed
- Age influences prognosis and treatment response
Approximate Synonyms
- Acute Lymphocytic Leukemia
- Lymphoblastic Leukemia
- Acute Lymphoblastic Lymphoma
- Precursor B-cell Lymphoblastic Leukemia
- Precursor T-cell Lymphoblastic Leukemia
Diagnostic Criteria
- Elevated white blood cell counts
- Predominance of lymphoblasts
- Low red blood cells
- Low platelets
- More than 20% lymphoblasts in bone marrow
- Presence of Philadelphia chromosome
- Flow cytometry for immunophenotyping
Treatment Guidelines
- Induction therapy with chemotherapy
- Supportive care including transfusions and antibiotics
- Continuation of chemotherapy in consolidation phase
- CNS prophylaxis with intrathecal chemotherapy
- Maintenance therapy with oral chemotherapy
- Regular monitoring for relapse and side effects
- Targeted therapy with tyrosine kinase inhibitors
- Monoclonal antibodies targeting CD19
- Stem cell transplantation with high-dose chemotherapy
Description
Related Diseases
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