Plasma cell leukemia

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ICD10 Diagnosis
Dx: Plasma cell leukemia
ICD10 code: C90.1
Pre-ICD10 counterpart: Multiple Myeloma
Charlson/ALERT Scale: Any malignancy, including lymphoma and leukemia, except of skin
APACHE Como Component: Immunocompromised
APACHE Acute Component: none
Start Date:
Stop Date:
External ICD10 Documentation

This diagnosis is a part of ICD10 collection.

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    • 2019-01-01
    • 2999-12-31
    • C90.1
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Additional Info

  • Plasma cell leukemia (PCL) is an aggressive form of multiple myeloma characterized by high levels of abnormal plasma cells circulating in the peripheral (circulating) blood. Normal plasma cells in the bone marrow produce antibodies that fight infection.
  • In myeloma most of the abnormal plasma cells remain in the bone marrow and are not found in the peripheral blood. In PCL, the abnormal plasma cells are in the peripheral blood. Therefore, PCL is considered to be an advanced form of myeloma. PCL can either originate as the primary manifestation of the disease (primary PCL with no history of myeloma) or as a transformation of myeloma (secondary PCL with progression of previously diagnosed myeloma).
  • Primary PCL is rare, with an estimated 1 per million of the general population diagnosed each year. Secondary PCL occurs in one to four out of 100 cases of myeloma and is becoming more common as myeloma patients are living longer.
  • As with myeloma, PCL is more common in African Americans than in Caucasians and is slightly more common in men than in women. As new insights and knowledge about the biology of myeloma and PCL are gained, it may be possible to determine which myeloma patients are at increased risk for developing PCL.
  • The causes of PCL are similar to those of myeloma. A series of genetic alterations during the development of a plasma cell may to lead to the cell’s uncontrolled growth. However, what triggers these alterations is not fully known. Risk factors, such as age and exposure to industrial and environmental elements, are thought to play important roles.

Leukemia vs Lymphoma

Any lymphoma may have a leukemic phase where the abnormal clonal cells appear in the circulation. Though this is sometimes referred to as a "leukemia", even by some oncologists, that is technically incorrect and ICD10 considers them to be lymphomas. This applies to essentially ALL so-called B-cell leukemias, which are actually lymphomas. It also applies to "NK-cell leukemia" which is also a lymphoma

As such, the following so-called "leukemias" should be coded as follows:

  • So-called B-cell leukemias describes several different types of lymphoid lymphomas which affect B cells -- and all these should be coded as Lymphoma, NOS
    • "B cell chronic lymphocytic leukemia"
    • "Precursor B cell lymphoblastic leukemia"
    • "Acute Lymphoblastic leukemia, mature B cell type"
    • "B cell prolymphocytic leukemia"
  • So-called "NK-cell leukemia" -- code as T-cell lymphoma
    • Aggressive NK-cell leukemia (also called aggressive NK-cell lymphoma, or ANKL), is a very rare type of NHL. The body makes large numbers of NK cells that are larger than normal. It is grouped with T-cell lymphomas.
    • There is a very rare slow-growing (indolent) type of NK-cell leukemia that has a more favorable prognosis. It is called chronic NK-cell leukemia and is treated like T-cell large granular lymphocytic leukemia.
  • The most common type of lymphoid leukemia is B-cell chronic lymphocytic leukemia.

Alternate ICD10s to consider coding instead or in addition

Leukemia codes:
Heme/immunology neoplasm codes:

Candidate Combined ICD10 codes

Related CCI Codes

Data Integrity Checks (automatic list)

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