Acquired disorders of the bone marrow: Difference between revisions

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{{CMG}}
'''''For the main page of bone marrow disorders click [[bone marrow disorders|here]].'''''<br>
'''''For congenital bone marrow disorders click [[congenital bone marrow disorders|here]].'''''
 
{{CMG}}; {{AE}} {{YD}}, {{Rim}}, {{SSK}}


==Overview==
==Overview==
Bone marrow disorders may either be congenital or acquired. Only acquired bone disorders are discussed below. Bone marrow disorders may be myelosuppression, myeloproliferation, or mixed/unclassified (features of both).
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==
==Classification==
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{{Family tree/start}}
{{Family tree/start}}
{{Family tree | | | | | | | | | | | |,| D01 | | | | | | | | D01= [[Myelodysplasia]]}}
{{Family tree |boxstyle= padding: 0; background: #DCDCDC; | | | | | | | | | | | | | |,| D01 | | | | | | | | D01= [[Myelodysplasia]]}}
{{Family tree | | | | | | | | | | | |!| | | | | | | | | | }}
{{Family tree | | | | | | | | | | | | | |!| | | | | | | | | | }}
{{Family tree | | | | | | | |,| C01 |+| D02 | | | | | | | | C01= All hematopoietic lineages| D02= [[Aplastic anemia]] ([[aplastic pancytopenia]])}}
{{Family tree |boxstyle= padding: 0; background: #DCDCDC; | | | | | | | | | |,| C01 |+| D02 | | | | | | | | C01= All hematopoietic lineages| D02= [[Aplastic anemia]] ([[aplastic pancytopenia]])}}
{{Family tree | | | | | | | |!| | | |!| | | | | | | | | | }}
{{Family tree | | | | | | | | | |!| | | |!| | | | | | | | | | }}
{{Family tree | | | | | | | |!| | | |`| D03 | | | | | | | | D03= [[Paroxysmal nocturnal hemoglobinuria]] ([[PNH]])}}
{{Family tree |boxstyle= padding: 0; background: #DCDCDC; | | | | | | | | | |!| | | |`| D03 | | | | | | | | D03= [[Paroxysmal nocturnal hemoglobinuria]] ([[PNH]])}}
{{Family tree | | | |,| B01 |(| | | | | | | | | | | | | | | B01= Myelosuppressive disorders}}
{{Family tree |boxstyle= padding: 0; background: #66CDAA; | | | | | |,| B01 |(| | | | | | | | | | | | | | | B01= [[Myelosuppression]] }}
{{Family tree | | | |!| | | |!| | | |,| D04 | | | | | | | | D04= [[Pure red cell aplasia]] ([[erythroblastopenia]])}}
{{Family tree |boxstyle= padding: 0; background: #A9A9A9; | | | | | |!| | | |!| | | |,| D04 | | | | | | | | D04= [[Pure red cell aplasia]] ([[erythroblastopenia]])}}
{{Family tree | | | |!| | | |!| | | |!| | | | | | | | | | }}
{{Family tree | | | | | |!| | | |!| | | |!| | | | | | | | | | }}
{{Family tree | | | |!| | | |`| C02 |+| D05 | | | | | | | | C02= One lineage|D05= [[Neutropenia]] ([[granulocytopenia]] and [[agranulocytosis]]}}
{{Family tree |boxstyle= padding: 0; background: #A9A9A9; | | | | | |!| | | |`| C02 |+| D05 | | | | | | | | C02= One lineage|D05= [[Neutropenia]] ([[granulocytopenia]] and [[agranulocytosis]])}}
{{Family tree | | | |!| | | | | | | |!| | | | | | | | | | }}
{{Family tree |boxstyle= padding: 0; background: #FFA07A;| | | |,| A01 | | | | | | |!| | | | | | | | | | A01= '''[[Bone marrow suppression]]''' }}
{{Family tree | | | |!| | | | | | | |`| D06 | | | | | | | | D06= [[Amegakaryocytic thrombocytopenia]]}}
{{Family tree |boxstyle= padding: 0; background: #A9A9A9; | | | |!| |!| | | | | | | |`| D06 | | | | | | | | D06= [[Amegakaryocytic thrombocytopenia]]}}
{{Family tree | | | |!| | | |,| C03 | | | | | | | | | | | | C03= [[Polycythemia vera]]}}
{{Family tree |boxstyle= padding: 0; background: #FAEBD7; | | | |!| |!| | | |,| C22 | | | | | | | | | | | | C22= [[T-cell immunosuppression]]}}
{{Family tree | | | |!| | | |!| | | | | | | | | | | | | | | }}
{{Family tree |boxstyle= padding: 0; background: #66CDAA; | | | |!| |`| B11 |(| | | | | | | | | | | | | | | B11= [[Immunosuppression]]}}
{{Family tree | | | |!| | | |!| C04 | | | | | | | | | | | | C04= [[Essential thrombocytopenia]]}}
{{Family tree |boxstyle= padding: 0; background: #FAEBD7;| | | |!| | | | | |)| C23 | | | | | | | | | | | | C23= [[B-cell immunosuppression]]}}
{{Family tree | | | |!| | | |!| | | | | | | | | | | | | | | }}
{{Family tree | | | |!| | | | | |!| | | | | | | | | | | | | | | }}
{{Family tree | A01 |+| B02 |+| C05 | | | | | | | | | | | | A01= Acquired bone marrow disorders| B02= Myeloproliferative disorders| C05=[[Primary myelofibrosis]]}}
{{Family tree |boxstyle= padding: 0; background: #FAEBD7;| | | |!| | | | | |`| C24 | | | | | | | | | | | | C24= [[ NK cell immunosuppression]]}}
{{Family tree | | | |!| | | |!| | | | | | | | | | | | | | }}
{{Family tree | | | |!| | | | | | | | | | | | | | | | | | | | | }}
{{Family tree | | | |!| | | |!| C06 | | | | | | | | | | | | C06= [[Systemic mastocytosis]]}}
{{Family tree |boxstyle= padding: 0; background: #D8BFD8;| | | |!| | | | | |,| C03 | | | | | | | | | | | | C03= [[Polycythemia vera]]}}
{{Family tree | | | |!| | | |!| | | | | | | | | | | | | | }}
{{Family tree | | | |!| | | | | |!| | | | | | | | | | | | | | | }}
{{Family tree | | | |!| | | |`| C07 | | | | | | | | | | | | C07= [[Chronic myeloid leukemia]] ([[CML]])}}
{{Family tree |boxstyle= padding: 0; background: #D8BFD8;| | | |!| | | | | |)| C04 | | | | | | | | | | | | C04= [[Essential thrombocytosis]]}}
{{Family tree | | | |!| | | | | | | | | | | | | | | | | | }}
{{Family tree | | | |!| | | | | |!| | | | | | | | | | | | | | | }}
{{Family tree | | | |!| | | |,| C08 | | | | | | | | | | | | C08= [[Chronic myelomonocytic leukemia]] ([[CMML]])}}
{{Family tree |boxstyle= padding: 0; background: #D8BFD8;| | | |!| |,| B02 |+| C05 | | | | | | | | | | | | B02= <div style="background: #66CDAA;">[[Myeloproliferation]] </div>| C05=[[Primary myelofibrosis]]}}
{{Family tree | | | |!| | | |!| | | | | | | | | | | | | | }}
{{Family tree | | | |!| |!| | | |!| | | | | | | | | | | | | | }}
{{Family tree | | | |!| | | |)| C09 | | | | | | | | | | | | C09= [[Atypical chronic myeloid leukemia]] ([[aCML]])}}
{{Family tree |boxstyle= padding: 0; background: #D8BFD8;| | | |!| |!| | | |)| C06 | | | | | | | | | | | | C06= [[Systemic mastocytosis]]}}
{{Family tree | | | |`| B03 |(| | | | | | | | | | | | | | | B03= Mixed or unclassified}}
{{Family tree |boxstyle= padding: 0; background: #FFA07A;| A00 |+| A02 | | |!| | | | | | | | | | | | | | A00= '''Acquired bone marrow disorders''' | A02= '''[[Bone marrow proliferation]]'''}}
{{Family tree | | | | | | | |!| C10 | | | | | | | | | | | | C10= [[Juvenile myelomonocytic leukemia]] ([[JMML]])}}
{{Family tree |boxstyle= padding: 0; background: #D8BFD8;| | | |!| |!| | | |`| C07 | | | | | | | | | | | | C07= [[Chronic myeloid leukemia]] ([[CML]])}}
{{Family tree | | | | | | | |!| | | | | | | | | | | | | | }}
{{Family tree |boxstyle= padding: 0; background: #F0E68C;| | | |!| |!| | | |,|-|-|-|-| D07 | | | | | | | D07= [[T-cell lymphoproliferative disorder]]}}
{{Family tree | | | | | | | |`| C11 | | | | | | | | | | | | C11= [[Unclassifiable myelodysplastic/myeloproliferative disease]]}}
{{Family tree |boxstyle= padding: 0; background: #66CDAA;| | | |!| |`| B22 |(| | | | | | | | | B22= [[Immunoproliferation]]}}
{{Family tree |boxstyle= padding: 0; background: #F0E68C;| | | |!| | | | | |`|-|-|-|-| D08 | | D08= [[B-cell lymphoproliferative disorder]]}}
{{Family tree |boxstyle= padding: 0; background: #87CEFA;| | | |!| | | | | |,| C08 | | | | | | | | | | | | C08= [[Chronic myelomonocytic leukemia]] ([[CMML]])}}
{{Family tree | | | |!| | | | | |!| | | | | | | | | | | | | | }}
{{Family tree |boxstyle= padding: 0; background: #87CEFA;| | | |!| | | | | |)| C09 | | | | | | | | | | | | C09= [[Atypical chronic myeloid leukemia]] ([[aCML]])}}
{{Family tree |boxstyle= padding: 0; background: #FFA07A;| | | |`| A03 |-|-|(| | | | | | | | | | | | | | | A03= '''Mixed or unclassified''' }}
{{Family tree |boxstyle= padding: 0; background: #87CEFA;| | | | | | | | | |)| C10 | | | | | | | | | | | | C10= [[Juvenile myelomonocytic leukemia]] ([[JMML]])}}
{{Family tree | | | | | | | | | |!| | | | | | | | | | | | | | }}
{{Family tree |boxstyle= padding: 0; background: #87CEFA;| | | | | | | | | |`| C11 | | | | | | | | | | | | C11= [[Unclassifiable myelodysplastic/myeloproliferative disease]]}}
{{Family tree/end}}
{{Family tree/end}}
==Bone Marrow Suppression==
[[Bone marrow suppression]] may be any of the following:
* '''Myelosuppression''': Reduced number of [[myeloid]] lineage cells ([[RBC]], [[neutrophils]], [[basophils]], [[eosinophils]], [[monocyte]]s, [[platelets]], [[mastocytes]]).
* '''Immunosuppression''': Reduced number of [[lymphoid]] lineage cells ([[T-cell]], [[B-cell]], [[NK cell]]). However, NK cell deficiency is almost always congenital.
Since the majority of bone marrow suppression cases are [[myelosuppression]], the term “myelosuppression” is often synonymous with [[bone marrow failure|bone marrow suppression/failure]].


===Myelosuppressive Disorders===
===Myelosuppressive Disorders===
Myelosuppressive disorders are characterized by either pancytopenia or selective lineage deficiency caused by deficient hematopoiesis at the level of the bone marrow.  Bone marrow failure may be a result of a congenital disease or an acquired disease.
Myelosuppression is defined as bone marrow suppression of myeloid progenitor cells ([[RBC]], [[neutrophils]], [[basophils]], [[eosinophils]], [[monocyte]]s, [[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''====
====''All Hematopoietic Lineages''====


* Myelodysplasia (aka Myelodysplastic syndrome): A cause of bone marrow failure characterized by defective stem cells that fail to undergo normal maturation.  Bone marrow appears HYPER cellular in myelodysplasia.
* [[Myelodysplasia]] (aka [[myelodysplastic syndrome]]): A cause of [[bone marrow failure]] characterized by defective stem cells that undergoes abnormal maturation.  Bone marrow appears HYPERcellular in myelodysplasia.


* Aplastic anemia (aka Aplastic pancytopenia , bone marrow hypoplasia, hypoplastic bone marrow, bone marrow aplasia, aplastic bone marrow, ehrlich anemia): A cause of bone marrow failure that may be hereditary (Fanconi’s anemia) or acquired (drugs, chemicals, radiation, or viruses).  It is a distinct disease characterized by an “empty” bone marrow.  Pancytopenia is a manifestation of aplastic anemia. Unlike MDS, the bone marrow appears HYPOcellular in aplastic anemia. Aplastic anemia is not characterized by abnormal mature cells, and the few cells that mature are normal in aplastic anemia. Aplastic anemia is a misnomer since not only RBC (anemia) is affected.
* [[Aplastic anemia]] (aka [[aplastic pancytopenia]], [[bone marrow hypoplasia]], [[hypoplastic bone marrow]], [[bone marrow aplasia]], [[aplastic bone marrow]], [[ehrlich anemia]]): A cause of bone marrow failure that may be hereditary ([[Fanconi’s anemia]]) or acquired (drugs, chemicals, radiation, or viruses).  It is a distinct disease characterized by an “empty” bone marrow.  [[Pancytopenia]] is a manifestation of aplastic anemia. Unlike MDS, the bone marrow appears HYPOcellular in [[aplastic anemia]]. Aplastic anemia is not characterized by abnormal mature cells, and the few cells that mature are normal in aplastic anemia. Aplastic anemia is a misnomer since not only [[RBC]] ([[anemia]]) is affected.


* Paroxysmal nocturnal hemoglobinuria: Bone marrow failure characterized by clonal expansion of a mutated hematopoietic stem cell.
* [[Paroxysmal nocturnal hemoglobinuria]]: Bone marrow failure characterized by clonal expansion of a mutated hematopoietic stem cell.


====''Selective Hematopoietic Lineages''====
====''Selective Hematopoietic Lineages''====


* Pure red cell aplasia (aka erythroblastopenia): There is insufficient production of RBC characterized by absence or deficient erythroblasts in the bone marrow.
* [[Pure red cell aplasia]] (aka [[erythroblastopenia]]): There is insufficient production of RBC characterized by absence or deficient erythroblasts in the bone marrow.


* Neutropenia( aka granulocytopenia, granulopenia, agranulocytosis, agranulosis, neutrophilic leukopenia, neutrophilic leukocytopenia, neutrophilic leucopenia, neutrophilic leucocytopenia): Neutropenia is defined absolute neutrophil count < 1.5 x 109/L. Agranulocytosis is defined as severe neutropenia < 0.5 x 109/L. Although agranulocytosis and granulocytopenia should include reduced numbers of all granulocytes (either neutrophils, eosinophils, or basophils), the majority of cases of granulocytopenia are actually neutropenia since neutrophils constitute the majority of leukocytes; the term granulocytopenia almost always refers to deficient neutrophils.
* [[Neutropenia]] (aka [[granulocytopenia]], [[granulopenia]], [[agranulocytosis]], [[agranulosis]], [[neutrophilic leukopenia]], [[neutrophilic leukocytopenia]], [[neutrophilic leucopenia]], [[neutrophilic leucocytopenia]]): [[Neutropenia]] is defined as absolute neutrophil count < 1.5 x 10<sup>9</sup>/L. Agranulocytosis is defined as severe neutropenia < 0.5 x 10<sup>9</sup>/L. Although agranulocytosis and granulocytopenia should include reduced numbers of all granulocytes (either neutrophils, eosinophils, or basophils), the majority of cases of [[granulocytopenia]] are actually neutropenia since [[neutrophil]]s constitute the majority of leukocytes; the term [[granulocytopenia]] almost always refers to deficient neutrophils.


* Amegakaryocytic thrombocytopenia: Thrombocytopenia caused by a reduced number of megakaryocytes in the bone marrow.
* [[Amegakaryocytic thrombocytopenia]]: [[Thrombocytopenia]] caused by a reduced number of [[megakaryocyte]]s in the bone marrow.
 
===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 syndrome]]s may be classified as:
 
* [[T-cell immunodeficiency]] (e.g. [[AIDS]])
* [[B-cell immunodeficiency]] aka [[humoral immune deficiency]] (e.g. [[acquired hypogammaglobulinemia]])
* [[NK cell immunodeficiency]] (e.g. [[AIDS]])
 
==Bone Marrow Proliferation==
Bone marrow proliferation may be any of the following:
 
* '''Myeloproliferation''': Increased number of [[myeloid]] lineage cells ([[RBC]], [[neutrophil]]s, [[basophil]]s, [[eosinophil]]s, [[monocyte]]s, [[platelet]]s, and [[mastocyte]]s)
* '''Immunoproliferation''': Increased number of [[lymphoid]] lineage ([[T-cell]], [[B-cell]], [[NK cell]])


===Myeloproliferative Disorders===
===Myeloproliferative Disorders===
Malignant disease the 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).
[[Myeloproliferation]] is a malignant disease that causes abnormal growth of mature [[RBC]], [[WBC]], and [[platelet]]s. 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]]).
 
* [[Polycythemia vera]] (aka [[polycythemia rubra vera]], [[erythemia]], [[primary polycythemia]]): Bone marrow disease characterized by proliferation of RBC
 
* [[Essential thrombocytosis]] (aka [[essential thrombocythemia]], [[primary thrombocythemia]]): Bone marrow disease characterized by proliferation of platelets


* Polycythemia vera (aka polycythemia rubra vera, erythemia, primary polycythemia): Bone marrow disease characterized by proliferation of RBC
* [[Primary myelofibrosis]] (aka [[idiopathic myelofibrosis]], [[agnogenic myeloid metaplasia]]): Idiopathic replacement of bone marrow by fibrosis


* Essential thrombocytosis (aka essential thrombocythemia, primary thrombocythemiais): Bone marrow disease characterized by proliferation of platelets
* [[Systemic mastocytosis]]: Proliferation of [[mast cell]]s in bone marrow and other organs


* Primary myelofibrosis (aka idiopathic myelofibrosis, agnogenic myeloid metaplasia): Idiopathic replacement of bone marrow by fibrosis
* [[Chronic myelogenous leukemia]] (CML): Chronic proliferation of myelogenous leukemic blast cells


* Systemic mastocytosis: Proliferation of mast cells in bone marrow and other organs
===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 dyscrasia]]s, [[monoclonal gammopathy]], [[monoclonal gammopathy]] of undetermined significance, [[plasmacytosis]], [[plasmacytoma]], [[hypergammaglobulinemia]], [[paraproteinemia]], [[cryoglobulinemia]], [[plasma cell leukemia]], [[primary amyloidosis]], [[light-chain amyloidosis]], and [[AL amyloidosis]].


* Chronic myelogenous leukemia (CML): Chronic proliferation of myelogenous leukemic blast cells
==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.


===Mixed or Unclassified Disorders===
* Chronic myelomonocytic leukemia (CMML)
* Chronic myelomonocytic leukemia (CMML)


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* Juvenile myelomonocytic leukemia (JMML)
* Juvenile myelomonocytic leukemia (JMML)


* Unclassifiable Myelodysplastic (aka myeloproliferative disease)
* Unclassifiable myelodysplastic disease (unclassifiable myeloproliferative disease)


==Definitions of other Terms==
==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]).
* [[Pancytopenia]]: Reduced numbers of all 3 bone marrow cell types: [[RBC]]s + [[WBC]]s + [[platelet]]s. It is not a disease, but rather a lab finding that has several causes that may related to decreased bone marrow activity (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]])) or increased peripheral destruction or sequestration that is not related to the bone marrow (e.g. [[splenomegaly]] or [[hypersplenism]]).
* [[Leukopenia]]: Deficiency of absolute number of [[leukocyte]]s (generally either [[lymphocyte]]s or [[neutrophil]]s). The majority of cases of [[leukopenia]] are actually [[neutropenia]] since [[neutrophil]]s constitute the majority of [[leukocyte]]s.
** [[Neutrophilic leukopenia]] (aka [[neutrophilic leukocytopenia]], [[neutrophilic leucopenia]], [[neutrophilic leucocytopenia]], [[neutropenia]]): Absolute neutrophil count < 1.5 x 10<sup>9</sup>/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 [[lymphocyte]]s ([[T-cell]]s or [[B-cell]]s)
* [[Myelophthisic anemia]] (aka [[myelophthisis]]): A type of [[bone marrow failure]] or wasting caused by infiltration of abnormal non-marrow cells (e.g. tuberculous granuloma, [[fibrosis]], [[neoplasm]]).


* 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.
==References==
** Neutrophilic leukopenia / Neutrophilic leukocytopenia / Neutrophilic leucopenia / Neutrophilic leucocytopenia / Neutropenia: Absolute neutrophil count < 1.5 x 109/L
{{Reflist|2}}
** Eosinophilic leukopenia / Eosinophilic leukocytopenia / Eosinophilic leucopenia / Eosinophilic leucocytopenia / Eosinophilopenia: Reduced absolute number of eosinophils
** Basophilic leukopenia / Basophilic leukocytopenia / Basophilic leucopenia / Basophilic leucocytopenia / Basophilopenia: Reduced absolute number of basophils


* Lymphopenia / Lymphocytopenia: Reduced absolute number of lymphocytes (T-cells or B-cells)
[[Category:Hematology]]
* Myelophthisic Anemia / Myelophthisis: A type of bone marrow failure caused by invasion of abnormal constituents (e.g. fibrosis or tumor).

Latest revision as of 23:34, 20 August 2015

For the main page of bone marrow disorders click here.
For congenital bone marrow disorders click here.

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 suppression/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

References