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SOP Bulletin 219

ICD Coding

• ICD-9-CM Codes: 204.0
• ICD-10-AM Codes: C91.0

Brief description

This is a cancer of immature white blood cells (lymphoblasts).  There are two overlapping clinical presentations of the same disease - leukaemia, when bone marrow blast cells predominate, and lymphoma, when a mass lesion in the mediastinum or elsewhere is the predominant feature.

Confirming the diagnosis

Diagnosis is usually suspected based on a finding of swollen lymph nodes or a blood test showing abnormal white cells.  A bone marrow biopsy is required to confirm the diagnosis and differentiate the condition from other diseases.

The relevant medical specialist is a haematologist.

Diagnoses covered by SOP

• acute lymphoid leukaemia
• acute lymphocytic leukaemia
• B-lymphoblastic leukemia/lymphoma (B-ALL/LBL)
• T-lymphoblastic leukemia/lymphoma (T-ALL/LBL)
• lymphoblastic lymphoma

Conditions excluded from SOP

• acute myeloid leukaemia*
• acute undifferentiated leukaemia^#
• adult T-cell leukaemia/lymphoma* (non-Hodgkin lymphoma SOP)
• Burkitt’s lymphoma/leukaemia* (non-Hodgkin lymphoma SOP)
• chronic lymphoid leukaemia*
• chronic myeloid leukaemia*
• indolent T-lymphoblastic proliferation^#
• non-Hodgkin lymphoma*

* another SOP applies

^# non-SOP condition

Clinical onset

Clinical onset will be when the diagnosis was confirmed, or shortly before that time if characteristic symptoms were present, such as easy bruising/bleeding, recurrent infections or symptoms from anaemia. 75% of cases occur in children under 6, but there is a second peak in adults > 60 years old.

Clinical worsening

The only worsening factor is for inability to obtain appropriate clinical management.  The prognosis in adults is generally poor.  Worsening beyond the normal progression of the disease will be difficult to establish and will require specialist opinion.