Haematology Flashcards

Question

Types of Sickle-Cell Crises ??

Answer 1

Thrombotic 'Painful' Crises Sequestration Crises Aplastic Crisis Haemolytic Crisis Acute Chest Syndrome

Answer 2

Painful or Vaso-Occlusive Crisis - Ppt. bt Infection, Dehydration, Deoxygenation Dx. made Clinically Infarcts occur in various organs - AVN of Hip - Hand-Foot Syndrome in Children - Lung, Spleen, Brain

Answer 3

Sequestration Crisis - Sickling within organs (Spleen, Lungs) causes pooling of blood => Anaemia worsening - a/w Increased Reticulocyte count APLASTIC Crisis - Cause: PARVOVIRUS B-19 infection - Sudden fall in Hb - BM Suppression => REDUCED reticulocyte count

Answer 4

Vaso-occlusion within Pulm. Micro-Vasculature => Lung parenchyma Infarction - Dyspnoea, Chest pain, Pulm. infiltrates on CXR, Low pO2 MANAGEMENT - Pain relief - Resp. Support: O2 therapy - Abx.- Infection may ppt. Acute C S & C/F can be difficult to distinguish from pneumonia - Transfusion: improves Oxygenation

Answer 5

Acute Chest Syndrome

Answer 6

Abnormality in enzymes responsible for Biosynthesis of HAEM - Results in the overproduction of Intermediate compounds (Porphyrin) They are broadly classified into - ACUTE Porphyrias - CUTANEOUS Porphyrias

Answer 7

ACUTE Intermittent P (A D condition) - Porphobilinogen Deaminase (PBGD) deficiency 2) HEREDITARY Coproporphyria - Coproporphyrinogen Oxidase (CPOX) deficiency 3) VARIEGATE Porphyria - Protoporphyrinogen Oxidase defect 4) ALAD deficiency Porphyria (ADP) - Delta Aminilevulinic acid Dehydratase deficiency

Answer 8

1) PORPHYRIA CUTANEA TARDA (PCT) - Uroporphyrinogen Decarboxylase (UROD) deficiency 2) ERYTHROPOIETIC Protoporphyria & X-Linked Dominant Protoporphyria - EPP : FECH mutation => Ferrocheletase deficiency - XLDP : ALAS2 mutation => Gain in function mutation : Protoporphyrin synthesis exceeds the required amount [MC & More severe in MEN] - MC Porphyria in Children 3) CONGENITAL Erythropoietic P (CEP) - Uroporphyrinogen 3 Cosynthase (UROS) defect 4) HEPATOERYTHROPOIETIC Porphyria (HEP) - UROD deficiency - A R form of Familial PCT

Answer 9

Two types of PCT - PCT-1 : Sporadic or Acquired PCT - PCT-2 : Familial PCT MC is the Hepatic form (HEP) - UROD defect Caused by Hepatocyte damage - eg.- [-OH], Oestrogen Photosensitive Rash + Bullae, Skin Fragility on face & Dorsum of Hand Ix.- Urine: Uroporphyrinogen elevated Urine under Wood lamp: Pink florescence Rx.- CHLOROQUINE

Answer 10

A D condition - Protoporphyrinogen Oxidase defect Abd. signs + CHS c/f + Photosensitive Blistering Rash MC in South Africans

Answer 11

A D condition (PBGD defect) Toxic accumulation of - Delta-ALA & Porphobilinogen MC in Females in 20-40 yrs C/F Abdomen: Abd. pain, Vomiting CNS: Motor Neuropathy Psychiatric: Depression HTN & Tachycardia are common

Answer 12

Urine turns Deep Red on Standing - Raised Urinary Porphobilinogen (Elevated b/w attacks & to a greater extent during acute attacks) Assay RBCs for PBGD Raised Delta-ALA & Porphobilinogen Rx.- - Avoid Triggers Acute Attacks - IV Haematin or Haem Arginate - IV Glucose (if the above 2 are not immediately available)

Answer 13

RBCs fail to completely form Haem, whose biosynthesis takes place partly in Mitochondrion - This leads to Fe deposits in Mitochondria => forms a ring around the nucleus called Ring Sideroblast - Congenital or Acquired

Answer 14

Congenital Cause - Delta-ALA Synthase-2 deficiency Acquired Causes - Myelodysplasia - Alcohol - Lead - Anti-TB drugs

Answer 15

Hypochromic Microcytic Anaemia - MC in Congenital Iron Studies - High: Ferritin, Fe - High Transferrin Saturation Basophillic Strippling BM: Prussian blue stain shows Ringed Sideroblasts Rx.- Supportive - Treat the Cause - PYRIDOXINE may help

Answer 16

Along with AIP, Lead poisoning is considered if a combination of Abd. Pain + CNS signs are seen - Lead poisoning results in Defective Ferrochelatase & ALA Dehydratase function Features - Abd. Pain, - Constipation, - Fatigue - Peripheral Neuropathy (MC- Motor) - Neuropsychiatric features - BLUE Lines on Gum margins (20% cases & is very rare in Children)

Answer 17

Blood levels: > 10mcg FBC: Microcytic; Basophilic strippling & Clover-Leaf Morphology Raised S./ Urine Delta-ALA seen but difficult to differentiate b/w AIP Urine Coproporphyrin is increased Children: Lead accumulates in METAPHYSIS of bone Rx.- Chelating agents - Dimercaptosuccinic Acid (DMSA) - D-Penicillamine - EDTA - Dimercaprol

Answer 18

MC in Mediterranean & Africans Inherited in X-linked R form G6PD is the 1st step in Pentose Phosphate pathway which converts G6P ==> 6-Phosphogluconolactone - This reaction results in NADP==> NADPH - G6P + NADP => 6-Pgluconolactone + NADPH NADPH is imp. for converting oxidised Glutathione back to its reduced form REDUCED Glutathione protects RBCs from Oxidative damage by Oxidants such as Superoxide anion (O2-) & H2O2

Answer 19

Decreased G6PD => Decreased 'Reduced NADPH' => Decreased 'Reduced Glutathione' => Increased RBCs susceptibility to Oxidative Stress

Answer 20

Neonatal Jaundice often seen INTRAVASCULAR Haemolysis Gallstones are common Splenomegaly may be present Blood Film - HEINZ Bodies, Bites & Blister cells Dx.- G6PD Assay - Levels are checked around 3 months after an Acute Haemolysis episode - Older RBCs with severely Reduced G6PD activity will be haemolysed ==> So Reduced G6PD activity is NOT measured in the assay => FN results as newer reticulocyte-rich cells with higher G6PD activity are present

Answer 21

Anti-Malarials: Primaquine Ciprofloxacin Sulph-group drugs: Sulfanamides, SUs, Sulfasalazine Drugs that are SAFE - Penicillins - Cephalosporins - Macrolides - Tetracyclines - Trimethoprim

Answer 22

Males (X-L R) = Male & Females (AD) 2) African & Mediterranean descent = Northern European descent 3) Heinz bodies = Spherocytes (round, lack of central pallor) 4) G6PDd: Neonatal Jaundice, Infection or Drug ppt. Haemolysis, Gallstones 5) H Spherocytosis: Nn Jaundice, Gallstones, Splenomegay, Chr. C/F although haemolytic crises may be ppt. by infection

Answer 23

- Measure G6PD levels 3 months after an Acute attack - EMA Binding

Answer 24

MC in Northern European descent - A D defect in RBC Cytoskeleton - Normal Bi-Concave disc shape is replaced by a Sphere-shaped RBCs C/F - FTT - Jaundice, Gall stones - Splenomagaly - APLASTIC Crisis by Parvovirus - Variable degree of Haemolysis - MCHC & Reticulocytes: ELEVATED

Answer 25

1) FHx of HS + Typical C/F & Lab. Ix. (Spherocytes, Elevated MCHC & Reticulocytes) do NOT require any additional test 2) Equivocal Dx, do - EMA Binding test & Cryohaemolysis test 3) ATYPICAL Presentation - Electrophoresis Analysis of RBC membrane is the method of choice [Osmotic Fragility test was used in the PAST]

Answer 26

ACUTE Haemolytic Crisis - Supportive - Transfusion if necessary LONG TERM Rx - Folate replacement - Splenectomy

Answer 27

Hb has been oxidised from Fe2+ to Fe3+. This is Normally regulated by NADH Met-Hb reductase which transfers electron from NADH to Met-Hb => Reduction of M-Hb => Hb. - Tissue Hypoxia as Fe3+ cannot bind O2 - O2 curve is mover to LEFT

Answer 28

CONGENITAL - Hb chain variants: HbM, HbH - NADH Met-Hb Reductase deficiency ACQUIRED - Drugs: Sulfonamides, Nitrates (includes Recreational Nitrates- Amyl Nitrite 'Poppers'), Dapsone, Na Nitroprusside, Primaquine - Chemicals: Aniline Dyes

Answer 29

Chocolate Cyanosis Dyspnoea, Anxiety, Headache Severe: Acidosis, Arrhythmias, Seizures, Coma Normal pO2 but Decreased O2 sats. Rx.- - NADH Met-Hb Reductase deficiency: ASCORBIC Acid -Acquired cause- IV Methylthioninium Chloride (Methylene blue)

Answer 30

A R condition (Defect in DNA repair) - Increased risk of AML, Solid tumours Features Haematological: - Aplastic anaemia - Increased risk of AML CNS Skeletal abnormalities - Short stature - Thumb (absent or hypoplastic) or Radius abnormalities - Low set ears, Deafness, Strabismus Renal Abnormalities Skin Hypopigmentation - CAFE-AU-LAIT-Spots Rx.- BM TRANSPLANT

Answer 31

RELATIVE Causes - Dehydration - Stress: GAISBOCK Syndrome PRIMARY Cause - Polycythaemia Rubra Vera SECONDARY Cause - COPD - Altitude - OSA - Excessive EPO : Cerebellar Haemangioma, Hypernephroma, Hepatoma, Uterine Fibroids (can cause menorrhagia => blood loss, polycythaemia is rarely a clinical problem)

Answer 32

Red Cell Mass studies; In true P, the Total Red cell mass is - Males > 35 ml/kg - Females > 32 ml/kg

Answer 33

Myeloproliferative disorder due to Clonal proliferation of a marrow stem cell => increased RBC volume a/w overproduction of Neutrophils & Plt. - JAK2 mutation in 95% cases - Incidence peaks at 6th decade

Answer 34

Hyperviscosity Pruritus, typical after a Hot Bath Splenomegaly Haemorrhage (2ndary to Abnormal Platelet function) Plethoric appearance HTN in 1/3rd pts. Low ESR & Raised Leukeocyte Alk. P

Answer 35

BCHS recommends the following - FBC/Film (Raised Haematocrit; Neutrophils, Basophils, Platelets raised in 1/2 of pts.) - JAK2 mutation - Serum Ferritin - RFT, LFT If JAK2 mutation is (-)ve & No obvious 2ndary causes BCHS suggests the following tests - Red Cell mass - Arterial O2 Sats. - Abd. USS - S. EPO levels - BM aspirate & Trephine - Cytogenetic analysis - Erythoid Burst-forming unit (BFU-E) culture

Answer 36

1) JAK2 (+)ve PcRV : Dx. requires BOTH criteria to be present A1: High Haematocrit (Men >0.52 & Women >0.48) (OR) Raised Red Cell Mass (>25% above predicted) A2: JAK2 mutation

Answer 37

Requires A1 + A2 + A3 + Either another A or Two B criteria A1: Red cell mass >25% above predicted (OR) Haematocrit >0.60 in Men & >0.56 in Women A2 : Absent JAK2 mutation A3:No cause of 2ndary Erythrocytosis A4 : Palpable Splenomegaly A5 : (+)ve Acquired genetic abnormality (excluding BCR-ABL) in Haematopoietic Cell B1: Thrombocytosis (Plt. >450) B2: Neutrophil Leukocytosis (N >10 in Non-smokers; >12.5 in smokers) B3 : Radiological evidence of Splenomegaly B4: Endogenous Erythroid Colonies or Low Serum EPO

Answer 38

ASPIRIN - Reduces risk of Thrombotic events VENESECTION - 1st line Rx. to keep Hb in normal range CHEMOTHERAPY - Hydroxyurea (slight increased risk of 2ndary Leukaemia) - Phosphorus-32 therapy

Answer 39

Thrombotic events are a significant cause of Morbidity & Mortality - 5- 15% progress to Myelofibrosis - 5-15% progress to Acute Leukaemia (risk increased with CT)

Answer 40

Abnormally large & sticky multimers of vWF cause Plt. to clump within vessels - In TTP, there is a deficiency of ADAMTS13 (a Metalloprotease enzyme) which cleaves large multimers of vWF - Overlaps with HUS - More common in Adult FEMALE - Microthrombi & Ruptured RBCs (Clumps tearing RBCs apart)

Answer 41

CAUSES - Post-infection (UTI, GIT) - Pregnancy - Tumours, - SLE, - HIV - Drugs: Ciclosporin, OCPs, Penicillin, Clopidogrel, Aciclovir C/F - Fever - Fluctuating Neuro. signs (Microemboli) - MAHA, - Thrombocytopaenia - Renal Failure - Affects Small BV of Brain & Renals - Bleeding under skin

Answer 42

NO Antibiotics - Can worsen outcome ToC: PLASMAPHERESIS Steroids, Immunosuppressants Vincristine

Answer 43

Seen in YOUNG Children; Triad of - AKI, - MAHA, - Thrombocytopaenia Most cases are 2ndary cases (Typical HUS) - STEC O157:H7 (Verotoxigenic, Enterohaemorrhagic). This is the MCC in Children 90% cases - Pneumococcal infection - HIV - Rare: SLE, Drugs, Cancer

Answer 44

FBC: Anaemia, Thrombocytopaenia, Fragmented RBCs U&E: AKI Stool Culture - Look for STEC infection evidence - PCR for Shiga toxin

Answer 45

Supportive: Fluids, BT, Dialysis (if required) NO role of Abx. Plasmapheresis ONLY in - Severe + HUS not a/w Diarrhoea cases ECULIZUMAB (C5 inhibitor) - Greater efficacy than Plasmapheresis alone in Adult Atypical HUS Rx.

Answer 46

Immune mediated reduction of Platelet counts - Antibodies against [GP2b/3a] or [1b-V-IX] complex => Plt. destroyed Children: ACUTE Thrombocytopaenia after vaccination or infection Adults: tend to have a CHRONIC condition MC in Older Females

Answer 47

May be detected incidentally after a Routine Bloods Symptomatic Cases presents with - Petichae, Purpura - Bleeding (eg. Epistaxis) - Catastrophic bleed (eg.-Intracranial) is NOT a common presentation

Answer 48

1st line: Oral Prednisolone Pooled Normal Human IVIG - Used in Active Bleeds or if an Urgent Invasive procedure is necessary - Raises Plt. count quicker than Steroids Splenectomy is now less commonly used

Answer 49

ITP a/w AIHA Ix.- Anti-Platelet autoantibody (IgG) BM aspirate : Megakaryocytes in BM. - Should be carried out PRIOR to commencement of Steroids to rule out Leukaemia Rx.- - Oral PREDNISOLONE (80% respond) - Splenectomy if Plt. < 30 after 3 months of Steroid therapy - IVIGs - Immunosuppressants: Ciclosporin

Answer 50

HLH is an aggressive & potentially fatal syndrome of extreme immune dysregulation (MC in Children) - Occurs when certain Immune cells (Lymphocytes & Macrophages) become excessively activated => wide spread tissue inflammation & organ damage. Classified into 1) PRIMARY (Familial) HLH - Genetic form, manifests in Infancy or Early childhood, due to mutations is gene involved in immune regulation. (eg- PRF1, UNC13D) 2) SECONDARY (Acquired) HLH - Triggered by external factors, Infections (VIRAL, - EBV), Malignancy, Autoimmune disease, Rheumatologic

Answer 51

Failure in Cytotoxic activity of NK cells & Cytotoxic T lymphocytes (CTLs) results in Immune dysregulation by - Uncontrolled Immune activation: Macrophages & T-lymp. release high levels of Cytokines (IFN-gamma, TNF-Alpha, IL-6) ==> 'Cytokine Storm' - Systemic Tissue Damage: Excess Cytokine release, damages organs includine- Liver, Spleen & BM causing the hallmarks C/F of HLH In PRIMARY HLH, genetic mutation disrupts the mechanisms by which NK cells & CTLs kill infected or abnormal cells In SECONDARY HLH, immune Hyper-activation is triggered by External factors

Answer 52

Spectrum of C/F reflecting Systemic Inflammation Initially mistaken to Sepsis or other inflammatory disorders - Persistent Fever: Resistant to anti-pyretics - Hepatosplenomegaly a/w Abd. pain - Cytopaenias: Anaemia, Leukopenia, Thrombocytopaenia due to BM involved - CNS features: Headaches, Seizures, Altered Mentation - Rash & Jaundice (due to Systemic inflammation & Liver dysfunction)

Answer 53

5 of 8 clinical & lab. criteria set by the HLH-2004 guidelines 1) Fever > 38.5 C 2) Splenomegaly 3) Cytopaenias (affecting >= 2 cell lineages) 4) Hypertriglyceridaemia (Fasting TGs > 3.0 mmol/L) (OR) Fibrinogen < 1.5 g/L 5) HemoPhagocytosis in BM, Spleen, LN or Liver 6) HyperFerritinemia (> 500ng/ml) 7) Elevated Soluble CD25 (soluble IL-2 receptor) reflects T cell activation 8) Low NK cell activity, indicative of impaired immune regulation

Answer 54

Initial Work up: Lab. tests - Ferritin : Rapid elevation of Ferritin, typically > 10,000 ng/ml, is a hallmark but NOT specific to HLH - Triglycerides - LFTs - FBCs - Fibrinogen levels Bone Marrow Biopsy: essential for identifying HemoPhagocytosis Genetic Testing: Recommended for Primary HLH targeting common HLH- associated Gene mutation

Answer 55

Early & Aggressive Rx. is necessary to control Hyperinflammatory response & prevent organ failure 1) Initial Stabilisation - Supportive care: Fever Rx, IVF, correct cytopaenias - Treat the underlying Triggers: In Secondary HLH (Infection, Neoplasia, Autoimmune disorders) 2) PHARMACOLOGICAL Intervention - Dexamethasone (reduce inflam.) - ETOPOSIDE (anti-Ca drug, reduces Immune cell proliferation) - Severe Cases: CICLOSPORINE may be added IVIGs occasional added particularly in Infection-Triggered HLH 3) Primary HLH or Not responding to above Rx. => Haemopoietic Stem Cell Transplant (HSCT) is Curative - Usually started after achieving disease control with Chemotherapy

Answer 56

Depends heavily on EARLY Dx. & Rx - Without Rx., it is fatal - Secondary HLH: survival rates improve significantly - Primary HLH: Rx. id challenging, requires HSCT Primary HLH: Life long follow up Regular follow-up includes - Organ Function: LFT, RFT - Immune Surveillance: Regular blood counts & Inflammatory markers - BM Health: For those treated with HSCT, BM function assessment is critical

Answer 57

Primary HLH is mostly seen in Children & Secondary HLH is seen in all age groups HLH is MC in Children but Adult cases are increasing, particularly with Malignancy associated HLH

Answer 58

Abnormally high Platelet count > 400 Causes REACTIVE - Plt. are Acute phase reactants - Increases in response to Stress, severe infection, Sx - IDA can also cause a Reactive Thrombocytosis Malignancy ESSENTIAL Thrombosis - As a part of another MP disorder (CML, PcRV) - Hyposplenism

Answer 59

MP disorder which overlaps with CML, PcRV & Myelofibrosis - Megakaryocyte proliferation results in overproduction of Platelets Features - Plt. count > 600 - Both Thrombosis & Haemorrhage can be seen - "Burning Sensation in hand" - JAK2 mutation found in 50% cases Rx.- - Hydroxyurea (Hydroxycarbamide) - IFN-Alpha in younger pts. - Low dose ASPIRIN (reduces thrombosis risk)

Answer 60

MP Disorder (Fibrosis of BM) - Due to Hyperplasia of abnormal Megakaryocytes - Resultant release of Plt. Derived GF =(+)=> Fibroblasts - Haemotopoiesis develops in Liver & Spleen

Answer 61

Elderly with symptoms of Anaemia - Fatigue (MC symptom) - Massive Splenomegaly - Hypermetabolic C/F: Wt. loss, Night sweats, etc. Lab. Findings - Anaemia - Hign WBC & Platelet counts early in the disease - 'Tear-drop' poikilocytes - BM Biopsy: DRY Tap => TREPHINE Biopsy needed - High Urate & LDH (Increased cell turnover)

Answer 62

Malignancy of Lymphoid Progenitor cells affecting B or T cell lineage - Results in ARRESTING of Lymphoid cell maturation & proliferation of Immature blasts (Lymphoblast) ==> BM & Tissue infiltration MC Childhood Cancer Peak age: 2- 5 yrs 80% of Childhood leukaemias

Answer 63

GOOD Prognostic Factors - FAB- L1 type - Common ALL - Pre-B phenotype - Low Initial WBC - Del (9p) POOR Prognostic Factor - FAB-L3 type, - Male Sex - T or B cell Surface marker - Philadelphia T, t(9;22) - Age < 2yrs or > 10 yrs - CNS Involved, - Non-Caucasians - High Initial WBS (eg. > 100)

Answer 64

MC form of Acu. Leukaemia in adults - Seen as a Primary disease (OR) - Secondary transformation of MP disorder Features (Largely related to BM Failure) - Anaemia: Pallor, Lethargy, Weakness - Neutropenia: Even though WBC counts are very high, functioning N levels can be low; Frequent infections - Thrombocytopaenia - Splenomegaly - Bone Pain

Answer 65

> 60 yrs > 20% Blasts after 1st course of CT Cytogenetics: Chr. 5 or 7 deletion

Answer 66

- a/w t(15;17) which causes Fusion of PML & RAR-Alpha gene - Presents Younger than other types of AML (average= 25 yrs) - AUER Rods (On MyeloPeroxidase stain) - Heavy cytoplasmic Granulation Presentation - DIC or Thrombocytopaenia - Good Prognosis

Answer 67

M0 : Undifferentiated M1 : Without Maturation M2 : With Granulocyte maturation M3 : Acute Promyelocytic M4 : Granulocytic & Monocytic Maturation M5 : Monocytic M6 : Erythroleukaemia M7 : Megakaryoblastic

Answer 68

Monoclonal proliferation of Well-differentiated Lymphocytes - Almost always B-cells (99%) - MC leukaemia in Adults Features - Often none (Incidental finding) - Anorexia, Wt. loss - Bleeding, Infection - LNpathy more marked in CML

Answer 69

FBC: Lymphocytosis, Anaemia Smudge or Smear cells IoC: IMMUNOPHENOTYPING - CD5/ CD19/ CD23 positive

Answer 70

Progressive BM Failure - Devt. or Worsening of Anaemia &/or Thrombocytopaenia Massive (>10cm) or Progressive LNpathy Massive (>6cm) or Progressive Splenomegaly Progressive Lymphocytosis: - > 50% increase over 2 months (or) - Lymphocyte doubling time < 6m Systemic Symptoms - Wt. loss > 10% in past 6m - Fever > 38 C for > 2wks - Extreme Fatigue, Night Sweats - Autoimmune Cytopaenias (eg.-ITP)

Answer 71

Poor P factors (Median survival 3-5yr) - Male Sex - Age > 70yrs - Lymphocyte count > 50 yrs - Prolymphocytes comprising >10% of blood lymphocytes - Lymphocyte 2x time < 12 months - Raised LDH - CD 38 expression (+)ve - TP53 mutation - Chr. 17p deletion (5- 10%) Good Prognosis - Chr. 13q deletion; MC abnormality seen in 50% cases a/w good P

Answer 72

If NO indications for Rx - Monitor with regular FBCs 1st Line : Fludarabine, Rituximab, Cyclophosphamide (FCR) 2nd line: IBRUTINIB (used if FCR fails) Complications - Anaemia - HYPOGammaglobulinaemia => Recurrent infection - Warm AIHA (10-15% cases) => Rx.- Prednisolone - RICHTER'S Transformation (Convert to High-grade Lymphoma)

Answer 73

Occurs when Leukaemia cells enters LN & changes to High-grade, fast growing Non-HL - Pt. often becomes suddenly Unwell C/F (Indicated by 1 of the following) - LN Swelling - Fever without infection - Wt. loss - Night Sweats - Nausea - Abdominal Pain

Answer 74

Philadelphia Chr. is present in more than 95% pts - Translocation t[9(q34);22(q11)] - This results in ABL proto-oncogene from Chr 9 to fuse with BCR gene on Chr. 22 - This resultant BCR-ABL gene codes for fusion protein that has an excess of Tyrosine Kinase activity

Answer 75

Anaemia: Lethargy Wt. loss & Sweating Massive Splenomegaly: Abd. discomfort Increase in Granulocytes at different stages of maturation +/- Thrombocytosis DERCEASED Leukocyte Alk. P May undergo Blast transformation

Answer 76

AML in 80% cases ALL in 20% cases

Answer 77

1st line: IMATINIB Mesylate - Inhibitor of TK a/w BCR-ABL defect - Very High response rate in Chr. Phase CML Hydroxyurea IFN-Alpha Allogenic BM Transplant

Answer 78

Presence of Immature cells such as - Myeloblasts, Promyelocytes, Nucleated RBCs in peripheral blood - Due to BM infiltration causing Immature cells to be 'pushed out' (or) - Sudden demand for New cells CAUSES - Severe Infection - Severe Haemolysis - Massive Haemorrhage - Metastatic Ca with BM infiltration

Answer 79

The following helps in differentiating CML from LR 1) Leukaemoid Traction - High LAP - Toxic granulation (Dohle bodies) in WBCs - Left shift of Neutrophils ie <= 3 segments of nucleus 2) CML - Low LAP

Answer 80

Malignant proliferation disorder of B Cells - 4x MC in males Features - Pancytopaenia - Splenomegaly - Skin vasculitis in 1/3rd pts. - 'Dry tap' despite BM hypercellurity - Tartrate Resistant Acid Phosphatase (TRAP) stain (+)ve RX.- - 1st line: CT with Cladribine, Pentostatin - 2nd line: Immunotherapy with Rituximab, IFN-Alpha

Answer 81

Malignant proliferation of Lymphocytes characterised by presence of RS cells - Bimodal age distribution 3rd & 7th Decade Features - LNpathy (75%): Painless, Non-tender, Asymmetrical - Systemic (25%): Wt. loss, Pruritus, Night sweats, Fever (Pel-Ebstein) - Alcohol pain in HL - Normocytic anaemia, Eosinophilia - LDH is raised

Answer 82

NODULAR SCLEROSING - MC 70% cases - Good Prognosis - MC in Women, a/w Lacunar cells MIXED Cellularity (20% cases) - Good Prognosis - a/w Large no. of RS Cells Lymphocyte Predominant (5% cases) - BEST Prognosis Lymphocyte DEPLETED (Rare) - WORST Prognosis

Answer 83

POOR Prognosis 'B' Symptoms - Wt. loss > 10% in last 6 months - Fever > 38 C - Night Sweats Age > 45 yrs Stage 4 disease Hb < 10.5 g.dl Lymphocyte count < 600/ul or < 8% Male sex Albumin < 40g/l WBC >15,000/ul

Answer 84

Ann-Arbor Staging of HL 1 : Single LN 2 : >= 2 LN/ regions on I/L Diaphragm 3 : LN on Both sides of Diaphragm 4 : Spread beyond LNs Each Stage is divided into A or B A : No systemic C/F other than Pruritus B : (Poor Prognosis) - Wt. loss > 10% in last 6m, - Fever > 38 C, - Night sweats

Answer 85

Mainly done by BIOPSY but - LNpathy in HL can experience [-OH] induced pain in LN - B symptoms occurs Early in HL & Late in NHL - Extra-Nodal disease is much more common in NHL than in HL

Answer 86

Malignant proliferation of Lymphocytes which accumulate in LN & Other organs - Can affect either B or T cells & is further classified as High or Low grade - 6th MCC of Cancer in UK - NHL is much more common than HL Typically affects Elderly - 1/3rd cases in > 75 yrs old - MC in Men than in Women

Answer 87

- Elderly, - Caucasians - H/o Viral Fever (specifically EBV) - FHx, - H/o CT & RT - Chemicals (Pesticides, Solvents) - Immunodeficiency (Transplant, HIV, DM) - Autoimmune (SLE, Sjogren's, Coeliac's)

Answer 88

Painless LNpathy - Non-tender, Rubbery, Asymmetrical B Symptoms - Fever, Wt. loss, N sweats, Lethargy Extra-Nodal Diseases - Gastric: Dyspepsia, Dysphagia, Abd. Pain, - BM : Pancytopaenia, Bone pain - Lungs, - Skin, - CNS (Nerve Palsies) SIGNS - LNpathy: Cervical, Axillary, Inguinal - Palpable Abd. Mass: Hepatomegaly, Splenomegaly, LNs - Testicular Mass - Fever

Answer 89

1) Dx. IoC: Excision Node Biopsy - Certain types will have classical appearance of Burkitt's (Starry sky) 2) Staging: CT Chest, Abd., Pelvis 3) HIV testing 4) FBC & Blood Film (Normocytic anaemia, rules out Leukaemias) 5) ESR & LDH: (Prognostic Indicator) 6)LFT (if Liver metastases suspected) 7) PET-CT or BM Biopsy if Bone involved & 8) LP if CNS symptoms

Answer 90

Ann Arbor System 1 : 1 LN or 1 organ affected 2 : > 1 LN affected on I/L Diaphragm 3 : LN affected on Both sides above & below Diaphragm 4 : Extra Nodal Spread (eg.- Spleen, BM or CNS) +/- LN involvement Each stage is sub-divided into - A : If no 'B' symptoms seen - B : If 'B' symptoms (+)ve

Answer 91

1) Dependent on Subtype of NHL - Watchful wait/ CT/ RT 2) ALL pts. must receive Flu/ Pneumococcal vaccines 3) Antibiotics Prophylaxis : Pts. with Neutropaenia

Answer 92

BM Infiltration: Anaemia, Neutropaenia, Thrombpcytopaenia SVC Obstruction Metastasis Spinal Cord Compression Rx. related (S/E of CT) Prognosis - LOW Grade NHL : Better Prognosis - HIGH Grade NHL : Worse Prognosis but HIGH Cure rate

Answer 93

Low N counts < 1.5; Normal levels are 2.0 to 7.5 Subdivided into - Mild : 1.0 to 1.5 - Moderate : 0.5 to 1.0 - Severe : < 0.5

Answer 94

Viral: HIV< EBV< Hepatitis Drugs: Cytotoxics, Carbimazole, Clozapine Hamaetological Malignancy - Myelodysplastic malignancies - Aplastic anaemia Rheumatological conditions SLE : circulating Antineutrophil antibody RA : Hypersplenism in Felty's Severe Sepsis Haemodialysis

Answer 95

Common among Black Africans & Afro-Caribbeans ethnicity - Requires no Rx.

Answer 96

Relatively common complication of Cancer therapy, a consequence of Chemotherapy - Occurs 7- 14 days after Chemo Neutrophil < 0.5 in a pt. on Anti-Ca Rx & has one of the following - Fever > 38 C - Signs & C/F consistent with Sepsis

Answer 97

Prophylaxis - It is anticipated in Pts. on Chemo that N will go < 0.5 as a result of Rx. so FLUOROQUINOLONES are offered Rx - Abx. started immediately (do NOT wait for WBC results) - Empirical Abx.- TAZOCIN is started After Initial Rx., risk assessment & stratification is done to see if Rx. on OPD basis is possible - If pt. still Febrile & Unwell after 48 hrs, alternative Abx.- Meropenam +/- Vancomycin - If not responding after 4- 6 days: Ix for Fungal infection rather than starting on Antifungals - G-CSF is used in some pts.

Answer 98

High grade B cell Neoplasia There are 2 major forms - Endemic (African) form: Typically involves Maxilla or Mandible, EBV infection is strongly implicated - Sporadic form: Abdominal (Ileo-caecal) tumours are MC forms & is MC in HIV pts. a/w EBV to a lesser extent A/W c-myc gene translocation t(8;14) HP: 'Starry Sky' appearance: Lympho-cytes sheets interspersed with macrophages containing dead apoptotic tumour cells.

Answer 99

Chemotherapy (a/w Tumour Lysis S) - Rasburicase is given before CT Complications - Hyper: K+, PO4-, - Hypocalcaemia - Hyperuricaemia - Acute Renal Failure

Answer 100

Type of B-cell Lymphoma Genetics - CD5+, CD19+, CD22+, CD23-, CD10- - t(11;14) causing over-expression of Cyclin D1 (BCL-1) gene C/F: Widespread LNpathy - Poor prognosis

Answer 101

Is a Neoplasia of BM Plasma cells Peak incidence: 60- 70 yrs C/F 1) Bone disease: Bone pain - Osteoporosis + Pathological # (typically Vertebral) - Osteolytic lesions 2) Lethargy, Hyperviscosity, Infection 3) Hypercalcaemia - Primarily due to INCREASED Osteoclast bone resorption by local Cytokines (IL-1, TNF) released by Myeloma cells - Secondarily due to Impaired Renal function, Increased R-tubule Ca2+ reabsorption & elevated PTH-rP 4) Renal Failure 5) Amyloidosis eg.- Macroglossia, CTS, Neuropathy

Answer 102

Monoclonal proteins (IgG or IgA) in Serum & Urine- Bence J proteins BM: Increased Plasma cells Whole body MRI X-rays: 'Rain drop skull' random pattern of dark spots - NOTE: very similar to but subtly different from Pepper-pot skull seen in Primary HyperPTH

Answer 103

1M + 1m or 3m in pts. with C/F of MM MAJOR Criteria - Biopsy: shows Plasmacytoma - BM sample: 30% plasma cells - Elevated M proteins in blood/ urine MINOR Criteria - BM sample: 10%- 30% Plasma cells - Blood/Urine: Minor rise in M protein - Imaging: Osteolytic lesions - Blood: Low levels of Antibodies (not produced by cancer cells)

Answer 104

Raised B2-Microglobulin & Low levels of Albumin indicate POOR Prognosis Stage = Criteria = Median Survival - 1 = B2-M < 3.5mg/l & Albumin > 35 g/l = 62 months - 2 = Not Stage 1 or 3 = 45 months - 3 = B2-M > 5.5 mg/l = 29 months

Answer 105

COAGULATION Disorder [Intrinsic Pathway Coagulation defect] X-linked Recessive (No FHx in 30%) Dx. in Infant Males with Bleeding or prolonged BT INHERITED Haemophilia (X-l R) - MC form of Hamophilia ACQUIRED Haemophilia Due to Inhibitory Autoantibody a/w autoimmune disorder =(+)=> F-VIII inhibitors production Other causes - Idiopathic (50% cases) - Postpartum period - Malignancy (solid tumour or Lymphoproliferative disorder) - Medications

Answer 106

H- A (Classic H) - Clotting factor VIII deficiency H- B (Christmas disease) - Clotting factor IX deficiency H- C (Rosenthal Syndrome) - Clotting factor XI deficiency - A R inheritance - MC in Ashkenazi Jewish population

Answer 107

Within those categories, H can further be classified on Severity- - Mild: 5- 40% of normal Clotting Factor Levels (CFL) - Moderate: 1-5% of normal CFL - Severe (50% cases): < 1% of normal CFL Features - Haemoarthroses - Haematomas - Prolonged Bleeding after Sx. or Trauma

Answer 108

Suspected in YOUNG pts. with Prolonged Haemorrhage, Ecchymosis , or Haemarthrosis 1) Clotting Studies 2) Prolonged aPTT (hallmark) - Represents INTRINSIC pathway 3) Normal PT - Can rule out EXTRINSIC & COMMON coagulation pathway pathologies - Causes: Liver diseases, Vit.K def. or DIC 4) LFT (5) F-VIII & IX assay 6) Mixing studies (Sample plasma + Normal plasma for 2 hrs) 7) vWF antigen testing

Answer 109

MIXING Studies - [sample Plasma + normal Plasma] for 2 hrs - CF Deficiency: APTT should correct - CF Antibodies: APTT will not correct Clotting disorder can be due to - CF Deficiency or - Acquired CF inhibitors (MC against F-VIII

Answer 110

Prothrombin Time - Tests Common & Extrinsic pathway - Factors- 1, 2, 5, 7, 10 - PT: Play Tennis OUTside (Extrinsic) INR : Pt. PT/ Control PT - 1 = normal; > 1 = Prolonges PTT Tests - Tests Common & Intrinsic pathway - All Factors except: 7 & 13 - PTT: Play Table Tennis INside TT - Measures the rate of conversion or Fibrinogen to Fibrin - Prolonged by: AC, DIC, Liver disease, Hypofibrinogenemia

Answer 111

Acute episode of Mild H- A - Desmopressin (promotes vWF release) - CFs Conc. used only if Bleeding persists after DDAVP use Severe disease: IV CFs (prophylactically & Self administered) - Rx. goal: 30-50% of normal CFLs - 1-3 infusions/wk. for 45wks in 1 yr. REPLACE the missing CFs H- A : F-VIII replaced - Emicizumab H- B : F-IX replaced Over time, Antibodies can develop to the replacement CFs called- Factor Inhibitors - Higher doses of missing CFs are needed for the same effect - Inhibitors MC in A (30%) > B (3%). NSAIDs & Aspirin is AVOIDED

Answer 112

MC inherited BLEEDING Disorder - Maj. inherited in A D fashion - Type 3 vWD is A R fashion vWF is a large glycoprotein which forms massive multimers upto 1,000,000 Da in size - Promotes Plt. adhesion to damaged Endothelium - Carrier molecule for F-VIII

Answer 113

Type-1 (80% cases) - Partially reduced vWF Type-3 (A R form) - Total lack of vWF - Most severe form Type-2 (abnormal vWF form):- Defective Plt. adhesion due to Decreased molecular wt. of vWF - Type-2B : Pathological increase of vWF-Plt. interraction - Type-2M : Decrease in vWF-Plt. interaction (not related to wt. of vWF) - Type-2N : Abnormal Binding of vWF to F-VIII

Answer 114

Intrinsic Coagulation pathway defect - Decreased quantity/ func. of vWF => Elevated PTT (vWF carries F-VIII) - Defect in Plt. Plug formation: Low vWF => Defect in Plt-vWF adhesion Prolonged BT APTT may be prolonged F-VIII levels are moderately reduced Defective Plt. aggregation with Ristocetin

Answer 115

Mild bleed: Tranexemic acid Desmopressin : Induces vWF release from Weibel-Palade bodies in Endothelial cells F-VIII concentrate

Answer 116

- t(8;140) : c-myc activation - t(11;14) : cyclin D1 activation - t(11;18) - t(14;18) : BCL-2 activation - t(15;17) - t(9;22) : CML (BCR-ABL)

Answer 117

COCPs (3rd gen > 2nd gen) HRT - Higher risk in women on Oestrogen + Progestogen preparation than those taking O-only pills Raloxifene & Tamoxifene Antipsychotics (specially Olanzapine)

Answer 118

Femoral >>> Subclavian

Answer 119

1) Active Ca (On Rx./ within 6m/Palliative) : 1 2) Paralysis,Paresis or Recent Plaster Immobilisation of LL : 1 3) Recent bedridden for >= 3 days or Maj. Sx. in < 12 wks requiring GA or Regional Anaesthesia : 1 4) Localised tenderness along the Deep vein system distribution : 1 5) Entire Swollen leg : 1 6) Calf swollen >=3cm than normal : 1 7) Pitting edema in symptomatic leg:1 8) Collateral superficial veins (Non- Varicose) : 1 9) Previous documented DVT : 1 10) Alternative Dx. is at least as likely as DVT : (-2)

Answer 120

If 2-level DVT Well's score >= 2 Proximal Leg USS in < 4hrs (OR) D-dimer (if not done) + Interim Therapeutic AC + Scan in < 24hrs 1) If Scan (+)ve => DVT diagnosed 2)If Scan (-)ve => Do D-dimer (if not done) - If D-d (+)ve : Stop AC+ Repeat scan in 1wk => If repeat (+)ve => DVT dx. & If 2nd scan (-)ve => Consider alternate Dx. & Stop AC - If D-d (-)ve : Consider alternate Dx. & Stop AC

Answer 121

DVT Unlikely D-dimer with results in 4hrs (OR) Interim Therapeutic AC while waiting - If (+)ve => Follow Well's score >=2 DVT likely algorithm - If (-)ve => Consider alternative Dx.

Answer 122

Pregnancy is a Hypercoagulable state - Maj. happens in LAST Trimester The following changes occur - Increase in Factor 7, 8, 10 & Fibrinogen - Decrease in Protein S - Uterus presses on IVC => venous stasis in LL Rx.- - S/C LMWH - Warfarin CI

Answer 123

Acquired disorder characterised by predisposition to - Venous or arterial thromboses - Recurrent Fetal loss - Thrombocytopaenia It can occur as a Primary or Secondary disorder - MCC is SLE

Answer 124

In Pregnancy, the following can occur - Recurrent miscarriages - IUGR, - Pre-Eclampsia - Placental abruption - Pre-term delivery - VTE Rx.- - Low dose ASPIRIN : started as soon as pregnancy is confirmed on UPT - LMWH : once Fetal heart is seen on USS. This is usually discontinued at 34 wks POG - These interventions increase the live birth rate by 7x

Answer 125

Coagulation cascade activation yields Thrombin that converts Fibrinogen => Fibrin; stable Fibrin Clot being the final product of Homeostasis Fibrinolytic system breaks down Fibrinogen & Fibrin Fibrinolytic system activation generates Plasmin (in the presence of Thrombin), which lyse the fibrin clots Fibrinogen & Fibrin breakdown result in Fibrin Degradation product In Homeostasis, PLASMIN is critical, as it is the central proteolytic enzyme of coagulation

Answer 126

Process of Coagulation & Fibrinolysis are dysregulated => results in Wide spread Clotting with resultant bleeding - Critical mediator of DIC: Transmemb Glycoprotein (Tissue Factor: TF) - TF is present on surface of many cell types (including Endothelial cells, Macrophages, Monocytes) & is not normally in contact with general circulation, but is exposed to the circulation after Vascular damage - Eg.- Exposure to IL-1, TNF, Endotoxin => TF released TF is abundant in: Lungs, Brain, Placenta - Once activated, TF binds with CFs =(+)=> Extrinsic CP (via F-VIII) => subsequently (+) Intrinsic (12 to 11 to 9) CP

Answer 127

Typical blood picture includes - Decreased Platelets - Decreased Fibrinogen - Increased PT & APTT & BT - Increased Fibrinogen degradation products - Schistocytes due to MAHA

Answer 128

Sepsis Trauma Obstetric complications eg.- Amniotic fluid embolism or haemolysis, HELLP Malignancy

Answer 129

- PT is prolonged; APTT, BT, Plt.C are all normal - BT is prolonged; PT, APTT, Plt.C are all normal - APTT is prolonged, PT if often normal (may be prolonged); BT, Plt.C are normal - PT, APTT, BT are prolonged; Plt.C is Low

Answer 130

These result in Hypercoagulable states (increased tendency to develop Thrombosis) INHERITED Causes Gain in Func. Polymorphisms - F-5 Leiden (activated Protein-C resistance) is the MCC - PT gene mutation: 2nd MCC Deficiencies of Naturally occurring AC - AT-3 deficiency - Protein-C deficiency - Protein-S deficiency ACQUIRED Causes - APLS - Drugs: COCPs

Answer 131

Condition = Prevalence = RR of VTE F-5 Leiden (Heterozygous) = 5% = 4 F-5 Leiden (Homozygous)= 0.05% = 10 PT gene mutation (Heterozygous) = 1.5% = 3 Protein C deficiency = 0.3% = 10 Protein S deficiency = 0,1% = 5-10 AT-3 deficiency = 0.02% = 10-20

Answer 132

F-5 Leiden (activated Protein-C Resistance) is the MC inherited form of Thrombophilia in UK Gain in func. mutation in F-5 Leiden protein. => Mis-sense mutation => F-5 is inactivated 10x more slowly by activated protein C than normal - Guanine => Adenine DNA Point Mutation => Arg506Gln mutation - So F-5 Leiden or Activated Protein-C Resistance

Answer 133

Heterozygous: 4x- 5x increase in VTE Homozygous: 10x increase in VTE but prevalence is much lower at 0.05% Screening for F-5 Leiden is NOT recommended, even after VTE - Previous H/o VTE itself is a risk factor for further episodes; dictates specific Rx. rather than the particular thrombophilia identified

Answer 134

DVT Cerebral Vein Thrombosis Recurrent pregnancy loss MC in Caucasian descents

Answer 135

Autosomal Co-dominant condition Decreased ability to INACTIVATE F-5a & F-8a. Increased risk of Warfarin-induced Skin Necrosis. - "Together protein C Cancels & protein S Stops, coagulation"

Answer 136

VTE Skin necrosis following Warfarin initiation - When W is 1st Initated, protein C synthesis is reduced => Temporary Pro-Coagulant state => Thrombosis in venules => Skin Necrosis; This is normally avoided by concurrent Heparin administration

Answer 137

A D Inherited cause of Thrombophilia - AT-III (-) several CFs primarily: Thrombin, F-10, 9. It mediates the effects of Heparin AT-III deficiency comprises of Heterogenous group of disorders, with some pts. having a - Deficiency of normal AT-III (or) - Produce abnormal AT-III

Answer 138

Post-Splenectomy, Coeliac's - Target cells - Howell-Jolly bodies - Pappenheimer bodies - Siderotic granules - Acanthocytes

Answer 139

Recurrent VTE Arterial thromboses do occur but are uncommon NOTE: AT-III deficiency have a degree of resistance to Heparin anti-Xa; levels should be monitored carefully to ensure adequate AC Rx.- - VTE are treated with Lifelong WARFARIN - Pregnancy: HEPARINISATION - AT-III concentrates RR of VTE is 10-20

Answer 140

Target cells 'Pencil' poikilocytes Dimorphic film (Micro- & Macro- cytic) - If combined with B12 deficiency

Answer 141

- 'Tear-drop' poikilocytes - Schistocytes - Hypersegmented Neutrophils

Answer 142

Raised in - PcRV - Myelofibrosis - Leukaemoid Reaction - Infections, - Pregnancy, - OCPs - Steroids, - Cushing's syndrome Low in - CML - Pernicious Anaemia - PNH - Infectious Mononucleosis

Answer 143

Intravascular Haemolysis - Haptoglobulins binds to free Hb

Answer 144

Raised in - H Spherocytosis - AIHA (a/w Spherocytosis) Decreased in - Microcytic anaemia (eg IDA)

Answer 145

CMV is transmitted in Leucocytes. As most blood products (except Granulocyte transfusions) are now Leucocyte depleted, CMV (-)ve are rarely required Irradiated blood products are T-lymphocyte depleted & is used to avoid Transfusion associated GVHD (TA-GVHD) caused by the variable donor T lymphocytes

Answer 146

Situations that need BOTH CMV (-)ve & Irradiated blood product - Granulocyte transfusions - Intrauterine transfusions - Neonates up to 28 days post expected date of delivery Needs only CMV (-)ve product - Pregnancy: Elective transfusion during pregnancy (not during labour or delivery) Needs only Irradiated blood product - BM or Stem cell transplant - Immunocompromised (eg.- CT or Congenital) - Pts. with/ previous Hodgkin L Do not need any of the 2 - HIV

Answer 147

'Clinically significant' but without 'Major bleed' in pts. with PT ratio or APTT ratio of > 1.5 - Typically 150 to 220 ml - Used prophylactically in pts. under going Invasive Sx. where there is a significant bleeding risk Universal Donor of FFP is "AB Blood" as it lacks anti-A or anti-B antibodies

Answer 148

Contains conc. F-8, vWF, Fibrinogen, F-13 & Fibronectin produced by further processing of FFP - MC used to replace FIBRINOGEN - Vol.- 15- 20 ml only Indication: Clinically significant + No major Bleed + Fibrinogen < 1.5g/L Use: DIC, Liver failure, 2ndary to massive BT (Hypofibrinogaenaemia) - In Haemophiliacs (when specific factor NOT available) & in VWD - Prophylactically in Invasive Sx. with a risk of significant bleed + Fibrinogen < 1.0 g/L

Answer 149

Used in emergency reversal of AC in pts. with either - Severe bleed (OR) - Suspected Intracranial Bleed Used prophylactically in pts. under going emergency Sx.

Answer 150

Indications Plt. Count < 30 + Bleeding grade 2 (eg.- Haematemesis, Malaena, prolonged epistaxis) Severe Bleed (Bleeding grade 3 or 4) OR Bleeding at critical sites line CNS + Plt.C < 100 Plt. transfusion has the HIGHEST risk of Bacterial Contamination Prophylactically given in Thrombo-cytopaenia before Sx./ Invasive procedure; Aim for a level of - > 50 for most pts. - 50- 75 in High risk bleeding pts - > 100 if Sx. at Critical sites

Answer 151

Threshold of 10 except where the Plt. transfusion is CI or there are alternative Rx. available Plt. Transfusion is CI in - Chr. BM failure - Autoimmune Thrombocytopaenia - HIT - TTP

Answer 152

It has been described in virtually every organ system: Biliary tree, Salivary glands, Periorbital tissues, Kidneys, Lungs, LNs, Meninges, Aorta, Breast, Prostate, Thyroid, Skin & Pericardium HP: Similar across organs, regardless of site - Analogous to Sarcoidosis - Raised IgG4 conc. in tissue & serum

Answer 153

Is a Rare type of Blood Cancer WM is seen Older Men - Lymphoplasmacytoid malignancy characterised by secretion of a Monoclonal IgM pataprotein

Answer 154

Reidel's Thyroiditis Autoimmune Pancreatitis Mediastinal & Retroperitoneal Fibrosis Periaortitis/ Periarteritis/ Inflammatory aortic aneurysm KUTTNER'S Tumour (Submandibular glands) & Mikulicz Syndrome (Salivary & Lacrimal glands) Possibly Sjogren's & PBC

Answer 155

IGs which undergo reversible ppt. at 4 deg. C, dissolve when warmed to 37 deg. C - 1/3rd cases are Idiopathic Features - Raynaud's (only in Type-1) - Cutaneous: Vascular purpura, Distal ulcerations, Ulceration - Arthralgia - Renal involved (Diffuse GN)

Answer 156

Type-1 (25% cases) - Monoclonal- IgG or IgM - a/w: M Myeloma, Waldenstrom's M Type-2 (25% cases) - Mixed Mono- & Poly- Clonal; usually with RF - a/w: HCV, RA, Sjogren's, Lymphoma Type-3 (50% cases) - Polyclonal; a/w RF - a/w: RA, Sjogren's

Answer 157

Low Complement (especially C4) High ESR Rx.- - Immunosuppression - Plasmapheresis

Answer 158

aka Benign Paraproteinaemia & Monolonal Gammopathy - Causes Paraproteinaemia & is often mistaken to Myeloma - Around 10% pts. eventually develop Myeloma at 10 yrs, with 50% at 15 yrs

Answer 159

Monoclonal IgM Paraproteinaemia Wt. Loss, Lethargy Hyperviscosity : eg.- Vision probs - IgM pentameric configuration increases the viscosity Hepatosplenomegaly LNpathy Cryoglobulinaemia eg.- Raynaud's

Answer 160

Differentiating features are - Asymptomatic - NO Bone pain or Increased risk of infection - 10- 30% of pts. have Demyelinating Neuropathy Differentiating features from M Myeloma - Normal immune function - Normal Beta-2-microglobulin levels - Lower level of Paraproteinaemia than myeloma eg.- < 30g/l if IgG or < 20g/l if IgA - Stable level of paraproteinaemia - NO C/F of Myeloma (eg. Lytic features on x-ray or Renal disease)

Answer 161

Primary Immunodeficiency due to a combined B & T cell dysfunction - X-linked-Recessive - Mutation in WASP gene Features - Recurrent Bacterial infection - Eczema - Thrombocytopaenia (very few Plt.) - Low IgM levels

Answer 162

Myelofibrosis CML Visceral Leishmaniasis (Kala azar) Malaria Gaucher's Syndrome

Answer 163

- Portal HTN (Cirrhosis) - Lymphoproliferative disease eg.- CLL, Hodgkin's L - Haemolytic Anaemia - Infection: Hepatitis, Glandular Fever - Infective Endocarditis - Sickle Cell (Maj. have an atrophied spleen due to repeated infarction) - Thalassemia - RA (Felty's)

Answer 164

Splenectomy Sickle Cell D Coeliac's, Dermatitis Herpetiformis Grave's SLE Amyloid Blood Film : Howell Jolly bodies, Siderocytes

Answer 165

aka Essential Thrombocythaemia, a Myeloproliferative disease - Thrombocytosis - Anaemia is present ONLY if there is bleeding - Raised Reticulocyte count Rx.- Hydroxyurea (Initial ToC) - 2nd line: Anagrelide in pts. > 60yrs, was inferior with respect to arterial thrombosis, maj. bleed & myelofibrotic transformation to H-urea - Anagrelide is better at reducing VTE than H-urea

Answer 166

CML (elevated WBCs) Myelofibrosis (causes Pancytopaenia)

Answer 167

Sub-type of Myelodysplasia that is a/w Raised Plt. count - Predominatly seen in women than in men

Answer 168

Pt. on Heparin, presents with DVT after a few days of starting > 50% fall in Plt. count Anti-PF-4 antibodies are responsible Stop Heparin based AC & start Non-Heparin based therapy