Tumors of the hematopoietic and lymphoid tissues (
American English
American English, sometimes called United States English or U.S. English, is the set of varieties of the English language native to the United States. English is the most widely spoken language in the United States and in most circumstances ...
) or tumours of the haematopoietic and lymphoid tissues (
British English
British English (BrE, en-GB, or BE) is, according to Oxford Dictionaries, "English as used in Great Britain, as distinct from that used elsewhere". More narrowly, it can refer specifically to the English language in England, or, more broadly ...
) are
tumors that affect the
blood,
bone marrow,
lymph, and
lymphatic system.
Because these tissues are all intimately connected through both the
circulatory system and the
immune system, a disease affecting one will often affect the others as well, making
aplasia
Aplasia (; from Greek ''a'', "not", "no" + ''plasis'', "formation") is a birth defect where an organ or tissue is wholly or largely absent. It is caused by a defect in a developmental process.
Aplastic anemia is the failure of the body to produ ...
,
myeloproliferation and
lymphoproliferation (and thus the
leukemias and the
lymphomas) closely related and often overlapping problems.
While uncommon in solid tumors,
chromosomal translocation
In genetics, chromosome translocation is a phenomenon that results in unusual rearrangement of chromosomes. This includes balanced and unbalanced translocation, with two main types: reciprocal-, and Robertsonian translocation. Reciprocal translo ...
s are a common cause of these diseases. This commonly leads to a different approach in diagnosis and treatment of hematological malignancies.
Hematological malignancies are malignant
neoplasms
A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
("cancer"), and they are generally treated by specialists in
hematology
Hematology ( always spelled haematology in British English) is the branch of medicine concerned with the study of the cause, prognosis, treatment, and prevention of diseases related to blood. It involves treating diseases that affect the produc ...
and/or
oncology. In some centers "hematology/oncology" is a single subspecialty of
internal medicine while in others they are considered separate divisions (there are also surgical and radiation oncologists). Not all
hematological disorders
Hematologic diseases are disorders which primarily affect the blood & blood-forming organs. Hematologic diseases include rare genetic disorders, anemia, HIV, sickle cell disease & complications from chemotherapy or transfusions.
Myeloid
* Hemog ...
are malignant ("cancerous"); these other blood conditions may also be managed by a hematologist.
Hematological malignancies may derive from either of the two major
blood cell lineages:
myeloid
Myeloid tissue, in the bone marrow sense of the word '' myeloid'' ('' myelo-'' + '' -oid''), is tissue of bone marrow, of bone marrow cell lineage, or resembling bone marrow, and myelogenous tissue (''myelo-'' + '' -genous'') is any tissue o ...
and
lymphoid cell lines. The myeloid cell line normally produces
granulocytes,
erythrocytes,
thrombocytes
Platelets, also called thrombocytes (from Greek θρόμβος, "clot" and κύτος, "cell"), are a component of blood whose function (along with the coagulation factors) is to react to bleeding from blood vessel injury by clumping, thereby ini ...
,
macrophages and
mast cells
A mast cell (also known as a mastocyte or a labrocyte) is a resident cell of connective tissue that contains many granules rich in histamine and heparin. Specifically, it is a type of granulocyte derived from the myeloid stem cell that is a par ...
; the lymphoid cell line produces
B,
T,
NK and
plasma cells. Lymphomas, lymphocytic leukemias, and myeloma are from the lymphoid line, while acute and chronic myelogenous leukemia,
myelodysplastic syndrome
A myelodysplastic syndrome (MDS) is one of a group of cancers in which immature blood cells in the bone marrow do not mature, and as a result, do not develop into healthy blood cells. Early on, no symptoms typically are seen. Later, symptoms may ...
s and myeloproliferative diseases are myeloid in origin.
A subgroup of them are more severe and are known as haematological malignancies (
British English
British English (BrE, en-GB, or BE) is, according to Oxford Dictionaries, "English as used in Great Britain, as distinct from that used elsewhere". More narrowly, it can refer specifically to the English language in England, or, more broadly ...
)/hematological malignancies (
American English
American English, sometimes called United States English or U.S. English, is the set of varieties of the English language native to the United States. English is the most widely spoken language in the United States and in most circumstances ...
) or blood cancer. They may also be referred to as liquid tumors.
Diagnosis
For the analysis of a suspected ''hematological malignancy'', a
complete blood count
A complete blood count (CBC), also known as a full blood count (FBC), is a set of medical laboratory tests that provide information about the cells in a person's blood. The CBC indicates the counts of white blood cells, red blood cells and pla ...
and
blood film
A blood smear, peripheral blood smear or blood film is a thin layer of blood smeared on a glass microscope slide and then stained in such a way as to allow the various blood cells to be examined microscopically. Blood smears are examined in th ...
are essential, as malignant cells can show in characteristic ways on
light microscopy. When there is
lymphadenopathy, a
biopsy
A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, or an interventional cardiologist. The process involves extraction of sample cells or tissues for examination to determine the presence or extent of a disea ...
from a
lymph node is generally undertaken
surgically
Surgery ''cheirourgikē'' (composed of χείρ, "hand", and ἔργον, "work"), via la, chirurgiae, meaning "hand work". is a medical specialty that uses operative manual and instrumental techniques on a person to investigate or treat a pat ...
. In general, a
bone marrow biopsy
Bone marrow examination refers to the pathologic analysis of samples of bone marrow obtained by bone marrow biopsy (often called trephine biopsy) and bone marrow aspiration. Bone marrow examination is used in the diagnosis of a number of conditio ...
is part of the "work up" for the analysis of these diseases. All specimens are examined microscopically to determine the nature of the malignancy. A number of these diseases can now be classified by
cytogenetics
Cytogenetics is essentially a branch of genetics, but is also a part of cell biology/cytology (a subdivision of human anatomy), that is concerned with how the chromosomes relate to cell behaviour, particularly to their behaviour during mitosis an ...
(AML, CML) or
immunophenotyping Immunophenotyping is a technique used to study the protein expressed by cells. This technique is commonly used in basic science research and laboratory diagnostic purpose. This can be done on tissue section (fresh or fixed tissue), cell suspension ...
(lymphoma, myeloma, CLL) of the malignant cells.
Classification
Historically, hematological malignancies have been most commonly divided by whether the malignancy is mainly located in the blood (
leukemia) or in
lymph nodes (
lymphomas).
Relative proportions of hematological malignancies in the United States
World Health Organization
4th Edition
NOS = "Not otherwise specified"
Treatment
Treatment can occasionally consist of "watchful waiting" (e.g., in
CLL) or symptomatic treatment (e.g.,
blood transfusion
Blood transfusion is the process of transferring blood products into a person's circulation intravenously. Transfusions are used for various medical conditions to replace lost components of the blood. Early transfusions used whole blood, but mo ...
s in
MDS). The more aggressive forms of disease require treatment with
chemotherapy
Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemother ...
,
radiotherapy,
immunotherapy and—in some cases—a
bone marrow transplant
Hematopoietic stem-cell transplantation (HSCT) is the transplantation of multipotent hematopoietic stem cells, usually derived from bone marrow, peripheral blood, or umbilical cord blood in order to replicate inside of a patient and to produ ...
. The use of
rituximab has been established for the treatment of B-cell–derived hematologic malignancies, including follicular lymphoma (FL) and diffuse large B-cell lymphoma (DLBCL).
In addition to cure-directed treatment, people can benefit from
self-care to manage symptoms. For example, aerobic exercise, such as
walking, can reduce
fatigue and feelings of
depression in people with hematological malignancies.
Follow-up
If treatment has been successful ("complete" or "partial remission"), a person is generally followed up at regular intervals to detect recurrence and monitor for "secondary malignancy" (an uncommon side-effect of some
chemotherapy
Chemotherapy (often abbreviated to chemo and sometimes CTX or CTx) is a type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) as part of a standardized chemotherapy regimen. Chemother ...
and
radiotherapy regimens—the appearance of another form of
cancer). In the follow-up, which should be done at pre-determined regular intervals, general
anamnesis is combined with
complete blood count
A complete blood count (CBC), also known as a full blood count (FBC), is a set of medical laboratory tests that provide information about the cells in a person's blood. The CBC indicates the counts of white blood cells, red blood cells and pla ...
and determination of
lactate dehydrogenase or
thymidine kinase
Thymidine kinase is an enzyme, a phosphotransferase (a kinase): 2'-deoxythymidine kinase, ATP-thymidine 5'-phosphotransferase, EC 2.7.1.21. It can be found in most living cells. It is present in two forms in mammalian cells, TK1 and TK2. Cert ...
in serum. Hematological malignancies as well as their treatments are associated with complications affecting many organs, with the lungs being frequently affected
Epidemiology
Taken together, haematological malignancies account for 9.5% of new cancer diagnoses in the United States and 30,000 patients in the UK are diagnosed each year.
Within this category, lymphomas are more common than leukemias.
See also
*
Myelodysplastic–myeloproliferative diseases
References
External links
{{Myeloid malignancy, us=y
Hematologic malignant neoplasms