Haematology 6

oelomar's version from 2016-03-31 19:14


Question Answer
What is hairy cell leukaemia (HCL)?An uncommon haematological malignancy characterised by an accumulation of abnormal B cells in the bone marrow and spleen.
What are the clinical features of hairy cell leukaemia?Clinical features include anaemia, fever and weight loss. Splenomegaly occurs in 80%, lymphadenopathy is uncommon.
How is hairy cell leukaemia treated?The purine analogues 2-chloroadenosine acetate (2-CDA) (cladribine) and pentostatin are used; complete remission is achieved in 90% with just one cycle of treatment. The remissions sometimes last for several years and patients can be retreated. Rituximab is used in resistant cases.
Prolymphocytic leukaemia (can be B-cell or T-cell) is a more aggressive form of chronic lymphocytic leukaemia. How is it treated?Treatment generally comprises chlorambucil, splenectomy may be indicated and fludarabine can be useful.


Question Answer
Very simply, what is Hodgkin’s lymphoma?A type of lymphoma in which the cancer originates from lymphocytes. Hodgkin’s lymphoma is characterised by the orderly spread of disease from one lymph node group to another and by the development of systemic symptoms with advanced disease.
A history of infectious mononucleosis due to infection by Epstein-Barr virus may increase the risk of developing Hodgkin’s lymphoma. True or false?True.
What age range(s) are more likely to be affected by Hodgkin’s lymphoma?There are two peaks in incidence, around 25-35 and 70-75 years of age.
What relevance do Reed-Sternberg cells have with Hodgkin’s lymphoma?Reed-Sternberg cells in a lymph node biopsy are a hallmark of Hodgkin’s lymphoma.
CD30 and CD15 are rarely expressed in Hodgkin’s lymphoma. True or false?False, they are almost always expressed.


Question Answer
“Classical” Hodgkin’s lymphoma (HL) represents 95% of cases of HL. What are its sub-divisions?Nodular sclerosing HL (70%); lymphocyte-rich HL (5%); mixed cellularity HL (25%).
What is nodular sclerosing Hodgkin’s lymphoma and which lymph nodes are primarily affected?This type typically affects young adults and demonstrates a nodular growth pattern with many fibrotic bands present. It involves particularly cervical and supraclavicular lymph nodes and the anterior mediastinum.
What is lymphocyte-rich Hodgkin’s lymphoma and which lymph nodes are primarily affected?This type typically presents at a higher median age and is characterised by an infiltrate of many small lymphocytes and Reed– Sternberg cells. It often occurs in peripheral lymph nodes.
What is mixed-cellularity Hodgkin’s lymphoma?Mixed-cellularity Hodgkin’s lymphomas have mixed cellularity with lymphocytes, eosinophils, neutrophils and histiocytes. Reed–Sternberg cells are present but no fibrotic bands.
“Nodular lymphocytic predominant” Hodgkin’s lymphoma (HL) represents 5% of cases of HL. Are CD30 and/or CD15 expressed as they in classical HL?No. Nodular lymphocyte-predominant HL contains malignant L and H cells (lymphocytic and/or histiocytic Reed–Sternberg cell variants, also called “popcorn” cells) which are negative for CD15 or CD30. However, CD20 is expressed.
What are the clinical features of Hodgkin’s lymphoma?Lymph node enlargement, most often of the cervical nodes – these are usually painless and with a rubbery consistency. The pattern of spread is usually contiguous (i.e. touching along a boundary or at a point). Enlargement of the spleen/liver. Systemic “B” symptoms: fever, drenching night sweats, weight loss of >10% bodyweight. Other constitutional symptoms, such as pruritus, fatigue, anorexia and, occasionally, alcohol-induced pain at the site of enlarged lymph nodes. Symptoms due to involvement of other organs (e.g. mediastinum – cough and breathlessness).
How is Hodgkin’s lymphoma treated?The treatment of choice for early stage HL is brief chemotherapy (ABVD) followed by involved field irradiation. Advanced disease is also curable for a significant portion of patients, and involves cyclical combination chemotherapy (BEACOPP) with irradiation at bulk sites.
What are the four “staging’s” for Hodgkin’s lymphoma?1- Confined to a single lymph node region. 2- Involvement of two or more nodal areas on the same side of the diaphragm. 3- Involvement of nodes on both sides of the diaphragm. 4- Spread beyond the lymph nodes e.g. liver or bone marrow.
In Hodgkin’s lymphoma, each stage is divided into “A” or “B”. What do these mean?A=No systemic symptoms other than pruritus. B=Presence of “B” symptoms – weight loss >10% bodyweight in 6 months, explained fever of >38 degrees, or drenching night sweats.


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Name some of the differences between Hodgkin’s and non-Hodgkin’s lymphomas.Hodgkin’s lymphoma is marked by the presence of Reed-Sternberg cells, which are mature B cells that have become malignant, are unusually large, and carry more than one nucleus. The first sign of the disease is often the appearance of enlarged lymph nodes. Non-Hodgkin’s lymphoma, by contrast, can be derived from B cells or T cells and can arise in the lymph nodes as well as other organs. Both diseases are relatively rare, but non-Hodgkin’s lymphoma is more common. The median age of patients with non-Hodgkin’s lymphoma is 60, but it occurs in all age groups. Hodgkin’s lymphoma most often occurs in people ages 15 to 24 and in people over 60. There are more than 60 distinct types of non-Hodgkin’s lymphoma, whereas Hodgkin lymphoma is a more homogeneous disease. The diseases often follow different courses of progression. Hodgkin’s lymphoma tends to progress in an orderly fashion, moving from one group of lymph nodes to the next, and is often diagnosed before it reaches an advanced stage. Most patients with non-Hodgkin’s lymphoma are diagnosed at a more advanced stage.
What is non-Hodgkin’s lymphoma?These are malignant tumours of the lymphoid system. Most are of B cell origin with 30% of T cell origin. The cause is unknown.
What are the clinical features of non-Hodgkin’s lymphoma?Peripheral lymphadenopathy – Most patients present with painless, superficial lymph node enlargement. Systemic symptoms (“B” symptoms) – Fever, sweats and weight loss. Extranodal presentation – May involve the gastrointestinal tract, lung, brain, testes, thyroid and skin. Abdominal involvement may reveal hepatosplenomegaly.
What investigation is carried out in order to distinguish between different types of non-Hodgkin’s lymphoma?Lymph node biopsy.
Is follicular lymphoma a common type of non-Hodgkin’s lymphoma?It is the second most common type of non-Hodgkin’s lymphoma.
What is the most common type of non-Hodgkin’s lymphoma?Diffuse large B-cell lymphoma.
What are the clinical features of follicular lymphoma?Most patients present feeling well but with painless lymphadenopathy. Investigation usually reveals multiple sites of disease: involvement of the bone marrow is common. Managed conservatively it is a remitting and recurring disease with a clinical course running over a median of 10 years during which there will be about three “episodes” of relapse.
What are the causes of death in follicular lymphoma patients?Resistant disease, transformation to diffuse large B-cell lymphoma, and effects of therapy.
How is follicular lymphoma managed?Allogeneic stem cell haematopoietic transplantation offers a possibility of a cure. Chemo-immunotherapy can be used – rituximab added to cyclical combination chemotherapy.


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What is lymphoplasmacytic lymphoma?Also known as Waldenström’s macroglobulinaemia, this is a cancer affected B cells which often presents with extensive bone marrow infiltration, and is almost the only lymphoma to be diagnosed on bone marrow biopsy alone. The main attributing antibody is IgM.
If left untreated, how likely diffuse large B cell lymphoma (DLBCL) to result in death?DLBCL is fatal without therapy within months. Patients present with rapidly progressive lymphadenopathy and progressive infiltration of many organs, e.g. spinal cord, gastrointestinal tract.
How is diffuse large B cell lymphoma treated?In the absence of relevant co-morbidity all patients should receive cyclical combination chemo-immunotherapy, the gold standard being cyclophosphamide, hydroxydaunorubicin, vincristine, prednisolone and rituximab (CHOP + R).
What is the prognosis of diffuse large B cell lymphoma?Progression during therapy or failure of the initial treatment to achieve complete remission has a very poor prognosis.


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What are the three types of Burkitt’s lymphoma?Endemic, sporadic, and AIDS-related.
What are the clinical features of Burkitt’s lymphoma?The endemic form is always EBV-associated, whereas the sporadic form is 30% EBV-related. The endemic form also occurs in equatorial Africa and corresponds to the distribution of malaria. The commonest presenting feature is a rapidly growing jaw tumour in a young child in the endemic form. Otherwise, the next most common is an abdominal mass often associated with bone marrow involvement.
What is the “starry sky” appearance seen on microscopy in Burkitt’s lymphoma?Sheets of lymphocytes with interspersed macrophages.
What are Sezary disease and mycosis fungoides?Both are cutaneous lymphomas in which T-cells are affected.


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What is multiple myeloma?A cancer of plasma cells, which accumulate in the bone marrow and therefore interfere with the production of normal blood cells.
What is a paraprotein?A protein found in the blood only as a result of cancer or other disease.
What is the Bence Jones protein, and what conditions is it relevant to?This is a monoclonal globulin protein found in the urine of patients affected by multiple myeloma or Waldenström’s macroglobulinaemia.
What age group is most likely to be affected by multiple myeloma?Myeloma is a disease of the elderly (median age = 60).
How can multiple myeloma affect the bone (not including the bone marrow)?Myeloma increases osteoclast activity leading to the typical lesions, usually seen in the spine, skull, long bones and ribs. Soft tissue plasmacytomas also occur and they are the usual cause of spinal cord compression in myeloma.
What is the most common feature of the bone disease seen in multiple myeloma?Bone pain – most commonly backache owing to vertebral involvement (60%).
How can multiple myeloma affect the bone marrow?The bone marrow is infiltrated with plasma cells, resulting in anaemia, neutropenia, thrombocytopenia, together with production of the paraprotein which may (rarely) result in symptoms of hyperviscosity (blood).
How can multiple myeloma affect the kidneys?Multiple myeloma can cause renal impairment, owing to a combination of factors – deposition of light chains in the renal tubules, hypercalcaemia, hyperuricaemia, use of NSAIDs and (rarely), deposition of amyloid.
How can multiple myeloma lead to recurrent infections?There is a reduction in the normal immunoglobulin levels (immuneparesis), contributing to recurrent infections, particularly of the respiratory tract.
What is the rouleaux formation seen in a blood film in multiple myeloma patients?Rouleaux are stacks or aggregations of RBCs which form because of the unique discoid shape of the cells in vertebrates. The flat surface of the discoid RBCs gives them a large surface area to make contact with and stick to each other; thus forming a rouleau. They occur when the plasma protein concentration is high, and because of them the ESR is also increased. Note that they are non-specific indicators of disease.
How is multiple myeloma diagnosed?Two out of three diagnostic features should be present: Paraproteinaemia or Bence Jones protein; radiological evidence of lytic bone lesions; an increase in bone marrow plasma cells. For symptomatic myeloma, evidence of end organ failure should also be present, i.e. anaemia, lytic lesions, renal impairment, hypercalcaemia, recurrent infections.
What is monoclonal gammopathy of unknown significance (MGUS) and what is its significance?MGUS describes an isolated finding of a monoclonal paraprotein in the serum, usually in the elderly; 30% go on to develop multiple myeloma.
How is multiple myeloma treated?Anaemia should be corrected. Bisphosphonates reduce progression of bone disease. The disease itself however, is incurable. In older or less fit patients, melphalan and prednisolone is the standard of care. In younger patients a dexamethasone based induction followed by melphalan with peripheral blood stem cell rescue (autotransplantation) has a significantly higher response rate. Thalidomide and its analogue lenalidomide and bortezomib are all used.