Tumours of Haematopoietic and Lymphoid Tissues: General Information
Essentials
The possibility of a malignant haematological disorder should be kept in mind when the patient's symptoms or signs include e.g. fever of unknown origin, increased susceptibility to infection, lymphadenopathy, hepatosplenomegaly, cytopenia of several cell lines, significant cytosis, or bleeding or thrombotic tendency.
Conditions requiring urgent or emergency treatment in hospital, such as aggressive lymphomas, acute leukaemias, complicated myeloma and myeloproliferative diseases should be recognized.
After treatments
follow-up for recurrence and late complications
appropriate palliative care, if needed
Signs and symptoms
Malignant blood diseases form a heterogeneous disease group. Tumours of the haematopoietic and lymphoid tissue encompass a total of approximately one hundred disease types.
The progress of some of these diseases is very slow, and they will neither produce symptoms nor require treatment for several months or years. Some of the patients will never need treatment.
Some of the diseases are, however, very aggressive and produce substantial local or systemic symptoms and are able to double the amount of diseased cells in a few days. Hospitalisation as an emergency or, in any case, urgently is often indicated.
thrombocytopenia (petechiae, proneness to bruising, bleeding)
anaemia (insufficient oxygen delivery to tissues, tiredness, dizziness, palpitations etc.).
The increasing leukaemic cell mass in the blood may cause hyperviscosity symptoms, and the infiltration of organs may produce local symptoms and dysfunction of the organs affected.
Leukaemias may be associated with
splenomegaly and hepatomegaly
lymphadenopathy
infiltrations, e.g. skin, gums and central nervous system.
In lymphomas Lymphomas, the symptoms are both local and systemic according to the disease type and the degree of infiltration:
lymphadenopathy
splenomegaly and sometimes hepatomegaly
systemic symptoms: fever, sweating, weight loss, itching etc.
an extranodal tumour: dysfunction of an organ affected by the tumour
In myeloproliferative diseases Polycythaemia Vera (PV)Myelofibrosis (Mf), the symptoms and organ damage are the result of increased blood volume and viscosity, arterial and venous occlusions as well as haemorrhages. The investigation of blood picture is especially important if occlusions occur in unusual locations but also in disturbances of cerebral or coronary circulation.
bone pain, bone erosion and fractures as well as hypercalcaemia
cytopenias
renal failure
susceptibility to infection.
Diagnosis and classification
The cornerstones of diagnosis
Full blood count
Microscopic morphology from MGG stained bone marrow and blood smears
In lymphomas, histological and immunohistological examination of the tumour
In plasma-cell diseases, the detection of M component by serum or urine protein fractionation or by serum free light chain assay as well as demonstration of plasma cell infiltration in the bone marrow or in the possible tumour. In suspicion of amyloidosis, the amyloid staining has to be ordered separately.
Criteria and classification of the diseases
The WHO classification of haematological malignancies is generally used. The classification is updated from time to time. The latest update is from 2016.
Immunologic (immunocytochemistry, flow cytometry) and enzymatic phenotyping of malignant cells (special stains)
Chromosomal G band, fluorescence in situ (FISH) investigation typing and molecular genetics to confirm chromosomal and gene changes
Staging
Malignant blood diseases are usually already widespread at diagnosis.
The degree of infiltration within the bone marrow is estimated with bone marrow biopsy and aspiration.
In Hodgkin's lymphoma and non-Hodgkin's lymphoma, prognosis and the choice of treatment is dependent on the extent of the tumour mass. Staging is carried out with the use of a CT scan and bone marrow biopsy. When investigating tumours that compress the brain and bone marrow, MRI scanning usually yields the most information. In gastrointestinal lymphomas, endoscopy is the investigation of choice, and endoscopic ultrasound examination is indicated in some cases.
Myeloma-induced bone changes are investigated with x-rays, and MRI scanning is usually required in spinal cord compression. CT scan is usable as well.
Urgency of diagnosis and treatment
Immediate admission to hospital (according to local guidelines to a hospital with appropriate diagnostic facilities):
maintenance of vital functions requires immediate measures (treatment of infections in immunocompromised patients, arresting bleeding, blood transfusion to ensure oxygen supply to tissues).
complications involving vital organs (for example, severe renal insufficiency or paraparesis in myeloma)
pathological fractures
thromboses, haemorrhage
suspected cases of acute leukaemia (clinical picture, blasts in the blood, cytopenia).
Hospital admission or admission to a specialized care clinic within 24 hours or at any rate within a few days:
suspected cases of fast growing lymphoma: systemic symptoms, rapidly progressing lymphadenopathy, hepatosplenomegaly or other tumour
severe cytopenia
hypercalcaemia
complicated cases.
Hospital admission within a few weeks (the patient may initially be managed by a primary care physician), for example:
monitoring of blood picture in a asymptomatic patient (chronic leukaemia, polycythaemia vera, essential thrombocythaemia)
monitoring of monoclonal gammopathy (monoclonal gammopathy of unknown significance, i.e. MGUS, myeloma) if the patient has none of the following: infections, severe cytopenia, bone pain, hypercalcaemia or renal failure.
monitoring an enlarged lymph node that is not progressing, or is associated with an infection, in a physically fit patient with few symptoms.
Treatment
Treatment modalities are very varied, change rapidly and require specialist expertise. The treatment is subtype specific, and targets are set taking the patient's general condition and treatment needs into consideration.
Destruction of malignant cells: chemotherapy, targeted drugs, radiotherapy, immunological treatment, high-dose treatment with stem cell transplantation support.
Supportive treatment: antimicrobials for an immunosuppressed patient's infections, blood transfusions, growth factors, general care of a cancer patient.
Treatment is carried out by a haematologist, oncologist, specialist in internal medicine or paediatric oncologist, as dictated by local policies.
The follow-up appointments and monitoring can be carried out by primary care physicians, in cooperation with the treating specialist centre.
Palliative care as indicated, arranged either by the primary health care team or the specialist centre.
Follow-up
The schedule and method of follow-up as well as the venue are decided on according to the disease type and treatment goals, and are arranged either by the primary health care team or the specialist centre.
The patient is observed for the recurrence of the disease or
emergence of late complications of the treatment.
Most patients who are in remission after treatment are able to work after a few weeks or months of convalescence.
References
Arber DA, Orazi A, Hasserjian R ym. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016;127(20):2391-405. [PubMed]