Hdp 3 Session 3 & 4 Haem & Lymph

Front 1

What is the basic function of lymphatic system

Back 1

Draining xs interstitial fluid and plasma proteins from tissue spaces. Transport dietary lipids from GIT to blood. Facilitating immune responses

Front 2

What is lymphadenopathy
Dev and CF

Back 2

Enlarged lymph nodes that become palpable and tender. Local: drainage of an inflammatory lesions near node. General: in presence of malignat or nonmalignant disease.

Front 3

What is Splenomegaly
Dev and CF

Back 3

Causes: infection (septic shock, glandular fever, Tb, malaria). Inflammation, haematological, portal hypotension
Invest: Ultrasound, CT

Front 4

What is Hypersplenism
Dev and CF

Back 4

Def: a condition characterised by exaggeration of the normal inhibitory or destructive fxs of the spleen, resulting in xu of the blood elements, singularly or in combinatorion, hypercellularity of the bone marrow, and sometimes splenomegaly.
Dev: result from splenomegaly, esp RA, portal hypertension, haemological do, lyphomas.
CF: causes Pancytopenia, haemolysis, Increase plasma volume.

Front 5

What are reasons for and consequences of splenectomy

Back 5

Immediate - increased platelet count (60-1000 for 2-3wks), long term = increased risk of overwhelming infections esp pneumococcal, carry an alert card.

Front 6

Define Leukaemia's

Back 6

Def: a group of neoplastic disorders involving WBCs. Leukaemia is characterised by distorted proliferation and development of leukocytes and their precursors in blood and Bone marrow.

Front 7

What is underlying pathology of Leukaemia's

Back 7

One or more of leukocyte types are present as undifferentiated, immature or no functional cells that multiply in the bone marrow. As they increase they infiltrate into the lymph nodes, spleen, liver, brain and other organs.
Acute: high prop of very immature blast cells in BM and peripheral circulatipon, onset abrupt.
Chromic: higher prop of mature cells (although have reduced fx), insidous onset, mild signs, better prog.
The proliferation of leukamic cells in the BM suppresses the production of other normal cells, leading to anaemia, thrombocytopenia, lack of normal leukocytes. Rapid turnover leads to hyperuricemia, kidney stones and renal failure. Crowding of cells in BM causes bone pain. Increase number of cells causes enlargement of lymph tissue.

Front 8

What is def, CF, Tx, Prognosis for ALL

Back 8

Def: malignancy of lymphoid cells, affecting B or T Lymphocyte cell lines, arresting maturation and promotion of uncontrolled proliferation of immature blast cells, with BM failure and tissue infiltration. Most common in children.
Classification 1. morphological (FAB), 2. Immunological; Precursor B-cell ALL, T-cell ALL, B-cell ALL. 3. Cytogenic: Chromosomal analysis predicts prognosis and recurrence.
S/S: Marrow failure (anaemia, low Hb), infections such as bacterial septicaemia, zoster, CMV, measiles, candidiasis, pneumonia. (low WCC), bleeding and bruising (low platelets). Infiltration (hepato-spleno-megaly, lymphadenopathy, CNS involvement.
Inv: FBC: Low Hb, High WCC, low platelets
ALL is defined as presence of over 30% lymphoblasts in BM.
Prognosis: 70-90% children, 35% adults. Poor prognosis if adult, male, Philadelphia chromosome.

Front 9

What is defn, CF, Tx, Prognosis for CML

Back 9

Uncontrolled clonal proliferation of myeloid cells. 40-60yrs, slight male predominance. Anaemia, night sweats, fever, weight loss, splenomegaly. blurred vision, headaches, gout, bleeding, bruising.
tests: increase WBC (>100x10to9th/L), Hb low or normal.Ph gene found on analysis. Hypercellular BM.
Prognosis: variable, survival 5-6yrs.
Tx: alpha-interferons induce heamatological remission, myeloablative therapy, transfusion.
Ph Chromosome present in >80%.

Front 10

What is epi, CF, Tx, Prognosis for CLL

Back 10

Uncontrolled proliferation and accumulation of mature B-lymphocytes
CF: recurrent infection, anaemia, bleeding, lymphadenopathy painless, splenomegaly.
Inv: Increased lymphocytes, FBC: low or normal Hb
Tx: Chlorambucil decreases lymphocyte count.Steroids.
Prognosis: 9-10yrs, infection predominant cause of death. Staging related to prognosis.

Front 11

Define and classify lymphomas

Back 11

Def: a lymphoma is a malignant neoplasm involving lymphocytes proliferation in the lymph nodes.
Class: abnormal proliferation of B or T lymphocyes; Histological appearance (hodgkins disease or non-HD); differentiated by lymph node biopsy; aggressive or indolent

Front 12

What is difference between lymphomas and leukamias

Back 12

Not always clear, depends on whether it presents primarily in lymph nodes or blood / BM.

Front 13

Define Hodgkin's disease

Back 13

Rare disease involving cells in lymphatic system to grow abnormally and spread in an orderly fashion over all the organs.
CF: painless enlarged non tender 'rubbery' lymph nodes, typically cervical. May have fever, night sweats, weight loss >10%, puritis, fatigue, anorexia, bone pain, jaundice. May have anaemia, spleno- hepato- megaly.

Front 14

What is Pathophysiology of HD

Back 14

Malignancy involves single lymph node (usually in the neck), then spread to adjacent nodes in ORDERLY fashion, and then to organs via the lymphatics. T-lympocytes appear defective and lymphocyte cound decreased. Presence of Reed-Sternberg cells.

Front 15

What is staging of HD

Back 15

Ann Arbor classification
Stage 1: single
Stage 2: affective 2 or more lymph nodes
Stage 3: cancer involves nodes in both sides of diaphragm and spleen
Stage 4: representative of diffuse extra lymphatic involvement eg bone, liver, lung.

Front 16

What are HD investigations

Back 16

FBC: normal or normochromic and normocytic anaemia. Raise ESR (inflammation), CXR may show mediastinal widening, CT scans reveal intrathoracic, abdo and pelvic lymphodenopathy. Lymph node biopsy showing Sternberg-Reed cells present.

Front 17

What is treatment for HD and prognosis

Back 17

Radiotherapy, cyclical combination of chemotherapy or both.
Relates to staging.

Front 18

What is Non-Hodgkins Lymphoma (NHL)

Back 18

Cells in lymphatic system grown abnormally and divide rapidly, forming lymphomas without Reed-Sternberg cells.
70% B cell origin. Divided into low grade and high grade reflecting rate of cell division and clinical progression. Subdivided into B or T cell origin (most are B cell type).

Front 19

What is cause, pathology and CF of NHL

Back 19

Congential immunodeficiency, acquired immunodeficiency, infection environmental.
Patho: Gene translocation
CF: nodal disease, superficial lyphoadenopathy, extranodal disease, systemic symptoms - night sweats, fever, weigt loss, pancytopenia due to marrow involvement

Front 20

What is treatment of NHL and prognosis

Back 20

Radiotherapy, chlorambucil.
76% with median survival of 9yrs. 4 factors correlate 1. histology, 2. late stage at presentation 3. increased serum lactate dehydrogrenase (by product of DNA breakdown), performance status.

Front 21

Define Myeloma

Back 21

A neoplastic disease involving the plasma cells (mature b-lymphocytes). An increased number of malignant cells replace the bone marrow and erode the bone. Blood cell production is impaired, as well as prodct of antibodies. Characterised by presence of paraprotein in serum. Onset 60yrs.

Front 22

What is pathology of myeloma

Back 22

An increase # of malignant cells replace BM and erod the bone. BC production is impaired, as well as prod of antibodies. Multiple tumours with bone destruction develop in the vertebra, ribs, pelvis, skull. Pathologic fractures common. Hypercalcaemia develops, tumour cells spread through the body, into lymp and infiltrate organs.
Test via urine excretion of bence-jones proteins.

Front 23

What are CF of myeloma

Back 23

Osteolytic bone lesions (unexplained bone pain, backache, fractures of long bones, vertebral collapse)
Hypercalcaemia
Anaemia, neutropenia, thrombocytopenia
Recurrent bacterial infections
Renal impairment

Disease based on presence of at least 2 of ; bence-jones proteins, radiological evidence of lytic bone lesions, increase in BM plasma cells.

Front 24

What Investigations do you do for myeloma

Back 24

FBC - normocytic anaemia, increase ESR or PV, bone scans show lytic lesions, BM aspirate shows infiltration by plasma cells.

Front 25

What is treatment for myeloma

Back 25

Correct anaemia, treat infection
Radiotherapy for bone pain, treat pathological changes. Generally incurable. Median survival is 3-4yrs.

Front 26

What is epidemiology of AIDS

Back 26

4th commonest cause of death world wide

Front 27

What is transmission of HIV

Back 27

Via semen, cervical secretions, blood. Vertical (mother - child) transmission most common route in children. Breast feeding 20% increased risk.

Front 28

Pathogenesis of HIV

Back 28

HIV particle binds to CD4 receptors on helper T-lyphocytes, monocytes, macrophages and neural cells. The HIV RNA is uncoated and released into cytoplasm.
Reverse transcriptase converts viral RNA to DNA, and is integrated into host genome. The copies are transcribed and translated and new virus particles are reassembled in the cytoplasm and budded out from the host cell. These virions infect new CD4 cells. As infection progresses depletion or impaired fx of CD4 +ve cells decreases immune function. Host is open to opportunist infection.

Front 29

Pathophysiology of HIV

Back 29

Retrovirus (sub family lentivirus) - characteristic possession of reverse transcriptase which allows viral RNA to be transcribed into DNA, which incorporates itself into host's genome.

Front 30

What are the stages of HIV infection

Back 30

Acute: often asymptomatic. Seroconversion may be accompanied by a transient illness 2-6 weeks after exposure (fever, malaise, myalgia). Asymptomatic infection follws.

Front 31

How do you diagnose HIV infection

Back 31

Via detection of virus-specific antibodies - Enzyme linked immunosorbent assay (ELISA) technique.

Front 32

What are CF of HIV

Back 32

Post infection - mild fllu like symptoms
Latent phase - if any CF, lymphadenopathy
Acute stage - GI effect ; wasting, anorexia, herpes simplex, candida,
Respiratory; TB, Pneumocystis carinii pneumona, Skin; kaposi's sarcoma, puritis, Neurological - h/a, photophobia, myelopathy, neuropathy, AIDS dementia complex, Haem: anaemia, lyphopenia, neutropenia, thrombocytopenia.

Front 33

What are opportunistic infections

Back 33

Immunodeficiency allows for infection by organisms not usually considered pathogenic. low CD4 count put pt at risk of AIDS defining OIs eg: TB, Pneumocystis carinii, candida, toxoplasmosis, strep pneumoniae.

Front 34

What is the monitoring regime of HIV

Back 34

Baseline investigations
Weight
HIV viral load
FBC
Liver and renal function
Absolute CD4 lymphocyte count and as %age of lypmphocytes. Falls as HIV progresses.

Front 35

What is management of HIV? Why does treatment fail?

Back 35

Multidisc approach. Highly active antiretroviral therapy (HAART).
Emergency of resistant strains, poor adherance, tolerance, ADRs.

Front 36

What is lymphodema?

Back 36

Chronic unilateral or bilateral oedema of the extremities due to accumulation of interstitial fluid. Is secondary to obstruction of lymph vessels or d/os of lymph nodes.

Front 37

What are CF of lymphodema?

Back 37

Present at birth (milroy's disease) or appears in adolescence. secondary due to obtruction.
CF: Insidious, noticable after exercise, exposure to warmth and premenstrual phase, erythema. Is chronic and non-pitting.
Rule out: soft pitting as in liver disease and cardiac failure.

Front 38

How to treat lymphodema

Back 38

Compression stockings, massage, if cellulitis use antibiotics, elevate limb at night. Avoid trauma as ulcerations can develop.

Front 39

What is splenomegaly?

Back 39

Caused by CML, myelofibrosis, malaria, inflammation (RA, SLE), haematological (haemolytic anaemia, leukaemias, lymphomas, myeloproliferatives), portal hypertension.

Front 40

What are investigations for splenomegaly?

Back 40

Ultrasound or CT. Spleen fx assesses by isotope scanning.

Front 41

What is hypersplenism

Back 41

Exaggeration of the normal inhibitory or destructive fxs of spleen, resulting in pancytopenia, anaemia, infection, bleeding.
Aet: splenomegaly. CF: pancytopenia, haemolysis, increase plasma volume. Tx: splenectomy if severe.

Front 42

Why do we do splenectomy?

Back 42

Trauma, autoimmune thrombocytopenic purpura, haemolytic anaemia, hypersplenism.
Probs: increase platelet count and thromboembolism (immed), increased risk of overwhelming infections.

Front 43

What is AML

Back 43

Neoplastic proliferation of blast cells derived from marrow myeloid elements. Rapidly progressive malignancy (death in 2 months if untreated, 20% 3 yr survival after chemo)

Front 44

What are symptoms of AML

Back 44

Marrow failure (anaemia, infection, bleeding), Infiltration (hepato - spleno-megaly, gum hypertrophy, skin involvement.

Front 45

How do you diagnose AML

Back 45

WCC increase, but can be normal or low. Diagnose by BM biopsy. Differentiation from ALL by microscopy: auer rods)

Front 46

What is etiology of leukaemia

Back 46

Chronic more common in older people, Acute in younger,
Inherited predisposisiton
Leukemic cells have chromosomal abnormality particl tranlocation (Philalephia chromosome #22 assoc with 97% CML and in ALL).