Acquired disorders of the bone marrow

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Yazan Daaboul, M.D., Rim Halaby, M.D. [2], Serge Korjian M.D.

Overview

Bone marrow disorders may be either congenital or acquired. Only acquired bone disorders are discussed below. Bone marrow disorders may be myelosuppression, myeloproliferation, or mixed/unclassified (difficult to classify or contains features of both).

Classification

Acquired bone marrow disorders include:

  • Bone marrow suppression: Reduced numbers of cells in bone marrow
  • Bone marrow proliferation: Increased numbers of cells in bone marrow
  • Mixed or Unclassified: Difficult to classify as suppression or proliferation at diagnosis or disorder that contains features of both.

Shown below is an algorithm that depicts the classification of acquired bone marrow disorders.

 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Myelodysplasia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
All hematopoietic lineages
 
 
Aplastic anemia (aplastic pancytopenia)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Paroxysmal nocturnal hemoglobinuria (PNH)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Myelosuppression
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Pure red cell aplasia (erythroblastopenia)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
One lineage
 
 
Neutropenia (granulocytopenia and agranulocytosis)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Bone marrow suppression
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Amegakaryocytic thrombocytopenia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
T-cell immunosuppression
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Immunosuppression
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
B-cell immunosuppression
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
NK cell immunosuppression
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Polycythemia vera
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Essential thrombocytosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Primary myelofibrosis
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Systemic mastocytosis
 
 
 
 
 
 
 
 
 
 
 
 
Acquired bone marrow disorders
 
 
Bone marrow proliferation
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Chronic myeloid leukemia (CML)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
T-cell lymphoproliferative disorder
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Immunoproliferation
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
B-cell lymphoproliferative disorder
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Chronic myelomonocytic leukemia (CMML)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Atypical chronic myeloid leukemia (aCML)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Mixed or unclassified
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Juvenile myelomonocytic leukemia (JMML)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Unclassifiable myelodysplastic/myeloproliferative disease
 
 
 
 
 
 
 
 
 
 
 
 

Bone Marrow Suppression

Bone marrow suppression may be any of the following:

Since the majority of bone marrow suppression cases are myelosuppression, the term “myelosuppression” is often synonymous with bone marrow failure.

Myelosuppressive Disorders

Myelosuppression is defined as bone marrow suppression of myeloid progenitor cells (RBC, neutrophils, basophils, eosinophils, monocytes, platelets, mastocytes). The majority of cases of bone marrow failure are due to myelosuppression. Myelosuppression results in either pancytopenia (deficiency of all lineages) or selective lineage deficiency.

All Hematopoietic Lineages

Selective Hematopoietic Lineages

Acquired Immunosuppression

Although immunosuppression is usually congenital, acquired immunosuppression (aka secondary immunosuppression, acquired immunodeficiency, secondary immunodeficiency, immunosuppressive disorder, immune deficiency disease) may be due to a disease of the bone marrow or the reticuloendothelial system. Bone marrow etiologies of acquired immunosuppression include: post-transplant immunosuppression, viral disease (eg HIV and AIDS), or drugs. Acquired immunodeficiency syndromes may be classified as:

Bone Marrow Proliferation

Bone marrow proliferation may be any of the following:

Myeloproliferative Disorders

Myeloproliferation is a malignant disease that causes abnormal growth of mature RBC, WBC, and platelets. Unlike myelodysplastic syndromes, myeloproliferative disorders are characterized by the presence of mature cells. Myelofibrosis is the end-stage of all myeloproliferative disorders and is characterized by replacement of bone marrow by fibrosis, which eventually results in a late state of myelosupression (compared with the the early state of myeloproliferation). All myeloproliferative disorders may potentially evolve and progress to acute myeloid leukemia (AML).

Immunoproliferative Disorders

Immunoproliferative disease (aka immunoproliferative neoplasm, immunoproliferative disorder) is a bone marrow disease that results in T-cell, B-cell, or NK cell proliferation. Examples include certain leukemias and plasma cell disorders (e.g. multiple myeloma, Waldenström macroglobulinemia). Synonyms and related diseases of B-cell lymphoproliferative disease include plasma cell dyscrasias, monoclonal gammopathy, monoclonal gammopathy of undetermined significance, plasmacytosis, plasmacytoma, hypergammaglobulinemia, paraproteinemia, cryoglobulinemia, plasma cell leukemia, primary amyloidosis, light-chain amyloidosis, and AL amyloidosis.

Mixed or Unclassified Disorders

Bone marrow disease that is difficult to classify at diagnosis or that includes features of bone marrow suppression and proliferation simultaneously.

  • Chronic myelomonocytic leukemia (CMML)
  • Atypical chronic myeloid leukemia (aCML)
  • Juvenile myelomonocytic leukemia (JMML)
  • Unclassifiable myelodysplastic disease (unclassifiable myeloproliferative disease)

Definitions of other Terms

  • Pancytopenia: Reduced numbers of all 3 bone marrow cell types: RBCs + WBCs + platelets. It is not a disease, but rather a lab finding that has several causes (e.g. pancytopenia may be due to insufficient production [aplastic anemia], inability of cells or mature [myelodysplasia], or replacement of normal bone marrow with fibrosis [myelofibrosis]).
  • Leukopenia: Deficiency of absolute number of leukocytes (generally either lymphocytes or neutrophils). The majority of cases of leukopenia are actually neutropenia since neutrophils constitute the majority of leukocytes.
    • Neutrophilic leukopenia (aka neutrophilic leukocytopenia, neutrophilic leucopenia, neutrophilic leucocytopenia, neutropenia): Absolute neutrophil count < 1.5 x 109/L
    • Eosinophilic leukopenia (aka eosinophilic leukocytopenia, eosinophilic leucopenia, eosinophilic leucocytopenia, eosinophilopenia): Reduced absolute number of eosinophils
    • Basophilic leukopenia (aka basophilic leukocytopenia, basophilic leucopenia, basophilic leucocytopenia, basophilopenia): Reduced absolute number of basophils
    • Lymphopenia (aka lymphocytopenia): Reduced absolute number of lymphocytes (T-cells or B-cells)
  • Myelophthisic anemia (aka myelophthisis): A type of bone marrow failure caused by invasion of abnormal constituents (e.g. fibrosis or tumor).

References