Plasma cell leukemia: Difference between revisions

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*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.
*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.
*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.
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== Alternate ICD10s to consider coding instead or in addition ==
== Alternate ICD10s to consider coding instead or in addition ==
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== Related CCI Codes ==
== Related CCI Codes ==
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== Related Articles ==
== Related Articles ==
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Latest revision as of 15:34, 12 September 2019

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.

  • SMW
    • 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.

Using ICD10 Malignancy Codes as a Comorbid Diagnosis

  • Any cancer/malignancy (either a "solid tumor" or a leukemia/lymphoma/bone marrow malignancy/"liquid tumor", i.e. any ICD10 code from C00-C99) can be a comorbid diagnosis --- BUT it's vital to distinguish malignancies in this category based on whether they are believed to be cured or not.
  • If it's still present (or believed to be present), then just include the code for the specific cancer as a comorbid diagnosis.
  • If INSTEAD, it's presumed cured, then in the "bin" of comorbid diagnoses combine the code for the specific cancer with this code: Past history, cancer (any type), believed cured
C00-C99 codes codes:
ICD10_codedescription
C14Lip or mouth or tonsils or pharynx, primary malignancy
C15Esophagus, primary malignancy
C16Stomach, primary malignancy
C17Small intestine, primary malignancy
C18Colon (large intestine), primary malignancy
C20Rectum or anus, primary malignancy
C22Liver, primary malignancy
C23Gallbladder, primary malignancy
C24Biliary tract, primary malignancy
C25Pancreas, primary malignancy
C26.1Spleen, primary malignancy
C26.9GI organ NOS, primary malignancy
C30Nasal cavity or sinus or middle ear, primary malignancy
C32Larynx, primary malignancy
C33Trachea, primary malignancy
C34Lung and/or bronchus, primary malignancy
C37Thymus, primary malignancy
C38.0Heart, primary malignancy
C38.3Mediastinum, primary malignancy
C38.4Pleura, primary malignancy
C39Respiratory system NOS, primary malignancy
C41Bone or cartilage (any location), primary malignancy
C43Skin, malignant melanoma
C44Skin NOS, primary malignancy
C45Mesothelioma (any site)
C46Kaposi's sarcoma
C47Nerves, peripheral or autonomic, primary malignancy
C48.1Peritoneal tissue/peritoneum, primary malignancy
C49Connective or soft tissue NOS (external or internal), primary malignancy
C50Breast, primary malignancy
C53Cervix, primary malignancy
C54Uterus, primary malignancy
C56Ovary, primary malignancy
C57Female genitalia NOS (internal or external), primary malignancy
C58Placenta, primary malignancy
C61Prostate, primary malignancy
C62Testis (testicular), primary malignancy
C63Male genitalia NOS (internal or external), primary malignancy
C64Kidney, primary malignancy
C66Ureter, primary malignancy
C67Bladder, primary malignancy
C68Urinary organ NOS, primary malignancy
C69Eye, primary malignancy
C70Meninges, primary malignancy
C71Brain, primary malignancy
C72.0Spinal cord, primary malignancy
C72.9Central nervous system NOS, primary malignancy
C73Thyroid gland, primary malignancy
C74Adrenal gland, primary malignancy
C75.0Parathyroid gland, primary malignancy
C75.1Pituitary gland, primary malignancy
C75.9Endocrine gland NOS, primary malignancy
C76.0Head and neck and face, primary malignancy
C76.8Primary malignancy (solid tumor) of site unknown or NOS
C77Lymph nodes, metastatic malignancy to them (also code primary site)
C78.0Lung, metastatic malignancy to it (also code primary site)
C78.2Pleura, metastatic malignancy to it (also code primary site)
C78.3Respiratory system NOS, metastatic malignancy to it (also code primary site)
C78.6Peritoneum, metastatic malignancy to it (also code primary site)
C78.7Liver or biliary system, metastatic malignancy to it (also code primary site)
C78.8Gastrointestinal system NOS, metastatic malignancy to it (also code primary site)
C79.0Urinary system NOS, metastatic malignancy to it (also code primary site)
C79.3Brain or meninges, metastatic malignancy to it (also code primary site)
C79.5Bone or bone marrow, metastatic malignancy to it (also code primary site)
C79.7Adrenal gland, metastatic malignancy to it (also code primary site)
C79.9Site NOS, metastatic malignancy to it (also code primary site)
C7A.0Malignant carcinoid tumor
C81Hodgkin's lymphoma (Hodgkin's disease)
C84T-cell lymphoma
C85.7Non-Hodgkin's lymphoma
C85.8Lymphoma, NOS
C88Malignant immunoproliferative disease
C90.0Multiple myeloma
C90.1Plasma cell leukemia
C90.21Plasmacytoma, NOS
C91.0Acute lymphoblastic leukemia (ALL)
C91.1Chronic lymphocytic leukemia (CLL)
C91.3Prolymphocytic leukemia
C91.4Hairy cell leukemia
C91.5T-cell leukemia
C91.7Lymphoid leukemia, NOS
C92.0Acute myeloid leukemia (AML)
C92.1Chronic myeloid leukemia (CML)
C92.4Acute promyelocytic leukaemia (APL)
C92.5Acute myelomonocytic leukaemia (AMML)
C93Monocytic leukemia, acute or chronic
C94.0Acute erythroblastic leukemia (erythroleukemia)
C94.2Acute megakaryoblastic leukemia
C95.0Leukemia, NOS
C96Malignancy (clonal disorder) of blood or lymphoid tissue, NOS

Metastases

Metastasis codes:

Regarding Presumptive Diagnosis of Malignancy

  • Rarely a presumptive diagnosis is made without any tissue confirmation. This generally occurs with:
    1. risk of obtaining tissue is very high
    2. plan would be palliative regardless
    3. patient would refuse care regardless.
  • Our issue for how to code a presumed malignancy without definitive histopathologic proof is this:
    • If the physicians are going to proceed with a treatment plan without that definitive histopathologic proof --- then code whatever is their best guess about what is present. Example: believed to be lung cancer with a big brain met, and they've decided NOT to do any biopsy but to give palliative radiation therapy, then you'd code lung cancer, and met to brain.
    • If the plan is to obtain a definitive histopathologic diagnosis soon or in the future, then instead code: Neoplasm of uncertain behavior (i.e. not clear if benign or malignant), NOS

"work-up for cancer"

If the cancer has not been confirmed then it should not be coded as cancer. Code relevant test abnormal test results or symptoms.

Testing codes:
Symptom/Sign codes:
Example:   

"I have a patient who comes in with vague respiratory and gi symptoms. They did a chest xray and found a lung mass. They are now working him up for a probable lung ca, with mets to various places. In the old coding I would use ca-nyd. I actually use the ca nyd subcode a lot. I’ve talked to you about this before, because there is no ca nyd in icd10. You told me that you either have cancer or you don’t. For this particular patient I really wouldn’t have anything else I could code in icd10 for him. His symptoms are extremely vague. I don’t really like coding just symptoms, if there isn’t a proper admit diagnosis that fits better anyway. I found a “neoplasm of uncertain behavior (i.e. uncertain if benign or malignant), nos”, but I don’t really like that one. It doesn’t really fit. Is it possible to get something like “admit for workup of malignancy”, or something along those lines?" (Debbie, 12:40, 2018 October 4 (CDT)) How should this be coded? Ttenbergen 12:40, 2018 October 4 (CDT)

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)

none found

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