Philadelphia chromosome positive acute lymphoblastic leukemia icd 10

Chapter 2 - Neoplasms (C00-D49) » Malignant neoplasms of lymphoid, hematopoietic and related tissue (C81-C96) » Acute lymphoblastic leukemia [ALL] (C91.0)

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Philadelphia chromosome positive acute lymphoblastic leukemia icd 10

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Philadelphia chromosome positive acute lymphoblastic leukemia icd 10

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Summary

Epidemiology

About 3,000 children in the United States and 5,000 children in Europe are diagnosed with ALL per year.

Clinical description

The peak incidence occurs between 2 and 5 years of age. ALL may be either asymptomatic or acute with a life-threatening haemorrhage, infection, or episode of respiratory distress. Although ALL primarily affects the bone marrow and peripheral blood, any organ or tissue may be infiltrated by the abnormal cells. The most frequent signs are lymphadenopathies, hepatosplenomegaly, fever, signs of haemorrhage, and bone pain.

Etiology

Most cases show chromosomal and genetic abnormalities, occurring spontaneously in genes playing important regulatory roles in controlling the lymphoid cell population. The most common ALL translocation, the t(12;21), appears to have good prognostic implications.

Diagnostic methods

ALL is biologically heterogeneous and morphologic, immunologic, cytogenetic, biochemical, and molecular genetic characterisation of leukaemia lymphoblasts is needed to establish the diagnosis or to exclude other possible causes of bone marrow failure and, finally, to classify ALL subtypes. Biological findings include hyperleukocytosis due to circulating lymphoblasts, anaemia and thrombocytopaenia. Diagnosis is established by bone marrow biopsy revealing leukaemic cell infiltration.

Management and treatment

The chemotherapy protocols adopted by international cooperative groups have four main objectives: induction with the aim of complete remission, preventative therapy to avoid central nervous system involvement, consolidation/re-induction, and maintenance therapy. Although management of relapse remains largely controversial, high dose chemotherapy blocks and stem cell transplantation are approaches increasingly adopted in most cases. In 2006, clofarabine obtained EU marketing authorisation as an Orphan drug for second line treatment of paediatric ALL patients. A novel tyrosine-kinase inhibitor, dasatinib, has been shown to be a safe and effective treatment option for adults with Philadelphia chromosome-positive acute lymphoblastic leukaemia and resistance or intolerance to imatinib. Clinical trials with this inhibitor are ongoing in the paediatric population. With the need to stratify patients into risk groups and to provide risk-adapted therapy, treatment requires high levels of organisation, expertise and knowledge.

Prognosis

The increase in the survival rate for children younger than 15 years of age has been dramatic, moving from less than 10% in the early 60's to about 75% in the late 90's. Unfortunately, however, results are still rather poor in countries with limited resources, which are home to around 80% of the child population.

Expert reviewer(s):  Pr Andrea BIONDI - Dr Roberto CHIESA - Dr C CITTERIO - Dr Valentino CONTER - Pr Carmelo RIZZARI - Dr Arturo SALA - Last update: August 2007

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.

What does Philadelphia chromosome positive mean?

Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) accounts for approximately one-fourth of cases of adult ALL. It typically presents with an aggressive clinical course, responds poorly to standard chemotherapy, and carries a high risk for relapse.

Does Philadelphia chromosome cause acute lymphoblastic leukemia?

The Philadelphia chromosome (Ph) is the most common cytogenetic abnormality in adult patients with acute lymphoblastic leukemia (ALL), occurring in about 20 % to 30 % of all cases [1–3].

Is Philadelphia chromosome curable?

With chemotherapy alone, only 20-30% of children with Ph+ ALL are cured. Allogeneic hematopoietic stem cell transplantation (HSCT) with a closely matched donor in first complete remission cures 60% of patients.

How do you code acute lymphoblastic leukemia?

ICD-9-CM Codes Lymphoid leukemia C91 (lymphoblastic leukemia) 204 (lymphoid leukemia) C91. 0 (acute lymphoblastic leukemia…) 204.0 (lymphoid leukemia, acute…)