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  2. ICD-10-CM Codes List
  3. C00–D49
  4. D37-D48
  5. Other neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue (D47)

Other neoplasms of uncertain behavior of lymphoid, hematopoietic and related tissue (D47)

The ICD-10 code D47 category covers various neoplasms of uncertain behavior of lymphoid, hematopoietic, and related tissue. These codes are used when a growth or abnormality arises from blood or immune system cells, but its exact nature; benign or malignant; is unclear. This category allows coding for conditions where clinical diagnosis is uncertain or evolving.

This section includes specific codes such as D47.01 for cutaneous mastocytosis, also known by terms like "bullous eruption" or "mastocytoma," and D47.02 for systemic mastocytosis, linked to “systemic mast cell disease.” Other important codes are D47.1 for chronic myeloproliferative disease, synonymous with disorders like chronic eosinophilic leukemia, and D47.2 for monoclonal gammopathy, a condition involving abnormal proteins also called “monoclonal gammopathy of uncertain significance.” Additionally, D47.Z1 represents post-transplant lymphoproliferative disorder (PTLD), a complication after organ transplantation characterized by abnormal lymphoid proliferation. These detailed ICD-10 codes guide accurate classification of uncertain hematologic and lymphoid neoplasms, assisting healthcare providers and coders in documentation and treatment planning.

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

Castleman Disease

Large benign, hyperplastic lymph nodes. The more common hyaline vascular subtype is characterized by small hyaline vascular follicles and interfollicular capillary proliferations. Plasma cells are often present and represent another subtype with the plasma cells containing IgM and IMMUNOGLOBULIN A.

Cytokines

Non-antibody proteins secreted by inflammatory leukocytes and some non-leukocytic cells, that act as intercellular mediators. They differ from classical hormones in that they are produced by a number of tissue or cell types rather than by specialized glands. They generally act locally in a paracrine or autocrine rather than endocrine manner.

Dysgammaglobulinemia

An immunologic deficiency state characterized by selective deficiencies of one or more, but not all, classes of immunoglobulins.

Mast Cells

Granulated cells that are found in almost all tissues, most abundantly in the skin and the gastrointestinal tract. Like the BASOPHILS, mast cells contain large amounts of HISTAMINE and HEPARIN. Unlike basophils, mast cells normally remain in the tissues and do not circulate in the blood. Mast cells, derived from the bone marrow stem cells, are regulated by the STEM CELL FACTOR.

Mast-Cell Sarcoma

A unifocal malignant tumor that consists of atypical pathological MAST CELLS without systemic involvement. It causes local destructive growth in organs other than in skin or bone marrow.

Mastocytoma

A solid tumor consisting of a dense infiltration of MAST CELLS. It is generally benign.

Mastocytoma, Skin

A variant of cutaneous mastocytosis which occurs as a single lesion usually in infants. It is found mostly in the wrist and trunk and there is no atypical cytomorphology.

POEMS Syndrome

A multisystemic disorder characterized by a sensorimotor polyneuropathy (POLYNEUROPATHIES), organomegaly, endocrinopathy, monoclonal gammopathy, and pigmentary skin changes. Other clinical features which may be present include EDEMA; CACHEXIA; microangiopathic glomerulopathy; pulmonary hypertension (HYPERTENSION, PULMONARY); cutaneous necrosis; THROMBOCYTOSIS; and POLYCYTHEMIA. This disorder is frequently associated with osteosclerotic myeloma. (From Adams et al., Principles of Neurology, 6th ed, p1335; Rev Med Interne 1997;18(7):553-62)

Primary Myelofibrosis

A de novo myeloproliferation arising from an abnormal stem cell. It is characterized by the replacement of bone marrow by fibrous tissue, a process that is mediated by CYTOKINES arising from the abnormal clone.

Schnitzler Syndrome

An extremely rare condition manifested as monoclonal IMMUNOGLOBULIN M dysproteinemia without features of lymphoproliferative disease, but with chronic urticaria, fever of unknown origin, disabling bone pain, hyperostosis, and increased erythrocyte sedimentation rate.

Thrombocythemia, Essential

A clinical syndrome characterized by repeated spontaneous hemorrhages and a remarkable increase in the number of circulating platelets.

Thrombocytosis

Increased numbers of platelets in the peripheral blood. (Dorland, 27th ed)