HEME Flashcards
1
Q
ANTI-Coagulation Pathologies (4)
A
- Hemophilia A + B
- Vitamin K Deficiency
- DIC
- Von Willebrand’s Disease
2
Q
Disorders of Primary Hemostasis (Platelets)
2 General Clinical Features?
A
- Mucosal Bleeding
2. Skin Bleeding
3
Q
Mucosal Bleeding
Pres (6)?
A
PRES:
- Epistaxis (nose bleed)
- Hemoptysis
- Hematuria
- Menorrhagia (heavy / prolonged periods)
- GI bleeding
- Intracranial bleeding (severe cases only)
4
Q
Skin Bleeding
Pres (2)?
A
PRES:
- Petechiae / Purpura / Ecchymoses
- Easy Bruising
5
Q
Petechia
Def’n, Causes (2)?
A
Small red or purple spots on skin caused by minor hemorrhage /
broken capillaries under skin.
6
Q
Purpura
Def’n?
A
Petechia 3-10 mm.
7
Q
Ecchymoses
Def’n?
A
Petechia > 1 cm.
8
Q
PRO-Coagulation Pathologies (4)
A
- Protein C/S Deficiency
- Antithrombin Deficiency
- Prothrombin Mutation
- Factor 5 Leiden
9
Q
Thrombotic Thrombocytopenic Purpura (TTP)
Pres (Pentad), Micro Finding?
A
PRES:
- Microangiopathic Hemolytic Anemia
- Thrombocytopenia
- Fever
- Neuro symptoms (thrombi involve CNS vessels)
- Renal symptoms (thrombi involve Renal vessels)
FINDING:
- Schistocytes
10
Q
Erythroblastosis Fetalis (Hemolytic Disease of Newborn)
Mech (4 steps), RX?
A
MECH:
Rh- mother exposed to Rh+ fetal blood (ie during delivery) ->
Mother makes Anti-Rh IgG ->
Anti-Rh IgG crosses placenta in subsequent pregnancy ->
Erythroblastosis Fetalis if fetus is Rh+.
RX:
- Rhogam = Rho(D) Ig
10
Q
Hemolytic Uremic Sx (HUS)
Mech, Causative Bugs (2)?
A
MECH = Toxin enhances cytokine release
CB:
- EHEC (-> Shiga-like toxin)
- Shigella (-> Shiga toxin)
11
Q
Disorders of Secondary Hemostasis (Coagulation)
2 General Clinical Features?
A
- Hemarthrosis (bleeding into joints + muscles)
2. Rebleeding after surgical procedures
12
Q
DIC
Causes (7: * “STOP Making New Thrombi” *),
DX, OTHER Labs (2),
Comp (2)?
A
CAUSES:
- Sepsis- Gram - (endotoxins + cytokines -> TF)
- Trauma
- Obstetric complications (Thromboplastin in amniotic fluid)
- Pancreatitis- Acute
- Malignancy (eg Mucin or granules, M3 AML)
- Nephrotic Sx (Loss of Antithrombin)
- Transfusion
DX:
-↑D-dimer
LABS:
- ↓Fibrinogen
- ↑Fibrin split products, esp D-dimer
COMP:
- Abruptio Placentae
- Waterhouse-Friderichsen Sx
13
Q
Trousseau Sx
Def’n, Causes (2 adenocarcinomas), Pres?
A
Hypercoagulability leading to Vasculitis + Migrating DVTs.
CAUSES:
- Lung adenocarcinoma
- Pancreas adenocarcinoma
PRES:
- Redness, tenderness + infl on palpation of extremities
14
Q
Raynaud Phenomenon
Def’n, Pres, Seen In?
A
Vasospasm in extremities.
PRES = Pale, cold + painful fingers / toes
SEEN IN:
- SLE
15
Q
Rouleaux Formation
Def’n, Mech, Seen In?
A
Stacks of RBCs.
MECH = due to↑ESR
SEEN IN:
- Multiple Myeloma
16
Q
Henoch-Schonlein Purpura
Def’n + 2 Locations Affected, Mech, Path (CLUE), Assoc,
Pres (Epi + 2 CLUES + 3)?
A
IgA vasculitis affecting skin + kidneys.
MECH = Vasculitis due to IgA deposition (= Immune-Complex mediated)
PATH: “Multiple GI lesions of SAME AGE”
ASSOC:
- Berger’s IgA Nephropathy (-> hematuria + F.S Glomerulosclerosis)
PRES: Child. ** Self-limited dz however may recur **
- “Palpable Purpura on buttocks / legs”
- Often “follows URI”
- Hematuria
- Ab pain + Melena
- Joint pain
17
Q
Fanconi Anemia
Def’n, Pres (2), Comp (2)?
A
Genetic loss of DNA CROSS-LINK REPAIR.
PRES:
- Short stature
- Aplastic Anemia
COMP:
- ↑incidence of tumors / leukemia
- Progression to AML
18
Q
“Anemia, Jaundice (UC Hyperbil),↑risk for bilirubin gallstones”
Micro Finding, Labs (2)?
A
EXTRAvascular Hemolysis (Hemolytic Normocytic Anemia)
FINDINGS:
- Spherocytes
LABS:
- LDH ↑
- UCB ↑
19
Q
“Target Cells +↓Haptoglobin”
Classification, Micro Findings (2), Labs (2), Urine?
A
INTRAvascular Hemolytic Anemia.
FINDINGS:
- ↑Reticulocytes
- Schistocytes
LABS:
- Haptoglobin ↓
- LDH ↑
URINE:
- Hb
20
Q
“Hemolytic Anemia in newborn”
A
PK Deficiency (EXTRAvascular Hemolytic Normocytic Anemia)
21
Q
“Inherited defect of RBC cytoskeleton-memb tethering proteins
(MC involving ankyrin, band + spectrin)” ->
MEMBRANE BLEBS”
A
Hereditary Spherocytosis
EXTRAvascular Hemolytic Normocytic Anemia
22
Q
“Red urine in morning”
A
PNH
23
Q
Aplastic Anemia
Def’n, Causes (5), Pres?
A
Damage to Hps Myeloid SC ->
Pancytopenia (esp anemia, neutropenia, thrombocytopenia).
CAUSES:
- Radiation
- Drugs
- Fanconi’s Anemia
- Viral agents (Parvo B19, EBV, HCV + HIV)
- Immune-mediated (primary SC defect)
PRES:
- Neutropenia
24
Iron Deficiency Anemia
Causes (4), Pres (3, incl 1 Triad)?
CAUSES:
* - Chronic bleeding
- CRC
- Malnutrition / malabsorption
- ↑Demand (eg pregnancy)
PRES:
- Fatigue
- Conjunctival pallor
- May manifest as Plummer-Vinson Sx:
1. Iron Def Anemia
2. Esophageal webs
3. Atrophic glossitis
25
Anemia of Chronic Disease (ACD)
Etiologies, Micro Finding, Labs, Course?
ET:
- Chronic infl
FINDINGS:
- Basophilic stippling
LABS:
- Hepcidin↑
COURSE = Can become MICROcytic in long-standing disease.
26
β-Thalassemia
Micro Findings (5: 3 general + 2 specific cell types)?
FINDINGS:
- Anisocytosis
- Poikilocytosis
- Hypochromia
- Schistocytes
- Target cells
27
β-Thalassemia Major (Homozygote)
Pres (3, incl 1 CLUE), Appearance on XR (CLUE)?
PRES:
- "Chipmunk facies"
- Severe anemia requiring bl transfusion (2ry hemochromatosis)
- Skeletal deformities (due to marrow expansion)
XR:
- "Crew cut" / "hair on end" appearance
28
Sideroblastic Anemia
Micro Findings (3), RX?
FINDINGS:
- Ringed Sideroblasts
- Pappenheimer Bodies
- Iron-laden mitochondria
RX = Vit B6
29
Cold Agglutinin Disease
Def'n, Seen In (3), Pres (CLUE)?
Autoimmune (IgM) ACUTE hemolytic anemia triggered by cold.
SEEN IN:
- CLL
- Mycoplasma pneumonia infections
- Infectious Mononucleosis
PRES:
- "BLUE painful fingers / toes"
30
G6PD Deficiency
Def'n, Mech (3 steps), Inher, Causative Agents (5),
Pres (Epi + CLUE + 1), Micro Findings (2)?
Hemolytic Anemia following oxidant stress.
MECH:
↓NADPH in RBCs -> Poor RBC defense against OXIDIZING agents ->
Hemolytic Anemia.
XR.
C.A: (Infection + Oxidizing agents)
- Infections (infl -> free radicals -> oxidative damage to RBCs)
- Fava beans
- Anti-TB drugs
- Primaquine
- Sulfa drugs
PRES: MC in Blacks
- "Back pain followed by hemoglobinuria a few days later"
- ↑malarial resistance
FINDINGS:
- Schistocytes
- Heinz Bodies + Bite Cells
31
Paroxysmal Nocturnal Hemoglobinuria (PNH)
Mech, Assoc, Pres (CLUE + Triad), Comp?
MECH = Fragile RBCs
ASSOC:
- DAF (GPI-anchored enzyme) def
PRES:
- "Red urine in MORNING"
- Triad:
1. Coombs- hemolytic anemia
2. Pancytopenia
3. Venous thrombosis
COMP:
-↑incidence of Acute Leukemia
32
Warm Agglutinin Disease
Def'n, Seen In (3: 2 dz + 1 drug), Micro Finding?
Autoimmune (IgG) CHRONIC hemolytic anemia. Coombs+.
SEEN IN:
- CLL
- SLE
- a-methyldopa
FINDING:
- Spherocytes
33
PK Deficiency
Pres (CLUE), Micro Finding?
PRES = "Hemolytic Anemia in a newborn"
FINDING:
- Schistocytes
34
HbC Defect
Micro Finding?
FINDING:
| - Target Cells
35
Hereditary Spherocytosis
Pres (2), Labs, RX?
PRES:
- Splenomegaly
- Aplastic crisis (Parvo B19 infection)
LABS:
- Osmotic Fragility Test +
RX = Splenectomy
36
Sickle Cell Anemia
Mech (3 factors that cause sickling + 2 results),
Pres (newborns), Labs, Appearance on XR (CLUE), DX,
Comp (6, incl 4 vaso-occlusive + 2 splenic + 2 renal),
RX (2)?
MECH:
Acidosis or Dehydration or↓O2 -> Anemia + Veno-Occlusive dz.
Newborns initially ASYMPTOMATIC due to↓HbS and↑HbF.
LABS:
- ESR↑
XR:
- "Crew cut" / "hair on end" appearance
DX:
- Hb Electrophoresis
COMP:
- Aplastic crisis (due to Parvo B19)
- Salmonella Osteomyelitis
- Painful vaso-occlusive crisis:
- Avascular necrosis
- Stroke
- Acute Chest Sx
- Dactylitis
- Spleen:
- Autosplenectomy
- Splenic sequestration crisis
- Renal:
- Papillary necrosis (due to low O2 in papilla)
- Microhematuria (due to infarcts in medulla)
- Priapism (due to sickled RBCs getting trapped in vasc channels)
RX:
- Hydroxyurea (↑HbF)
- BM transplantation
37
Angiopathic Hemolytic Anemias
Mech, Micro Finding?
MECH = Mechanical destruction of RBCs
FINDING = Schistocytes
38
Microangiopathic Hemolytic Anemia
Seen In (4: 2 heme + 2 other)?
SEEN IN:
- Malignant Htn
- SLE
- DIC
- TTP / HUS
39
Macrocytic Megaloblastic Anemias
Micro Findings (2)?
FINDINGS:
- Macro-Ovalocytes
- Hypersegmented Neutrophils
40
Pernicious Anemia
Causes?
CAUSES:
| - Autoimmune / Chronic Atrophic Gastritis
41
PAINLESS Lymphadenopathy (LAD)
Seen In (3)?
SEEN IN:
- Chronic infl
- Lymphoma
- Metastatic Carcinoma
42
Follicular Hyperplasia (B cell region)
Seen In (2)?
SEEN IN:
- Early HIV
- RA
43
Paracortex Hyperplasia (T cell region)
Seen In?
SEEN IN:
| - Viral infections
44
B SYMPTOMS of Lymphoma (3)
- Fever (low-grade)
- Weight Loss
- Night sweats
*** Same as TB ***
45
Nodular Sclerosis
Pres (CLUE), Micro Finding (CLUE)?
PRES = "Enlarging cervical or mediastinal lymph node in a young adult (usually female)"
FINDING:
- RS cells present in "lake-like" / "lacunar" spaces
46
Lymphocyte-Depleted HL
Seen In (2 Epi)?
SEEN IN:
- Elderly
- HIV + indivs
47
Mixed Cellularity HL
Micro Finding?
FINDINGS:
| - Abundant Eosinophils (RS cells produce IL-5)
48
Follicular Lymphoma
Def'n, B Cell Marker, Genetics, Pres (Epi + CLUE), RX, Comp?
Neoplastic prolif of small B cells (CD20+) -> Follicle-like nodules.
CD20+.
BCL2 (14;18) translocation.
PRES: Late Adulthood
- "Waxing + waning PAINLESS lymphadenopathy"
RX: *For patients who are symptomatic ONLY*
- Low-dose chemotherapy on Rituximab (anti-CD20 antibody)
COMP:
- Progression to Diffuse Large B Cell Lymphoma
49
Mantle Cell / Zone Lymphoma
Def'n, B Cell Markers (2), Genetics, Pres (Epi + 1)?
Neoplastic prolif of small B cells (CD20+) -> Expansion of mantle zone.
CD5+, CD20+.
Cyclin D1 (11;14) translocation.
PRES: Late Adulthood *Older Males*
- PAINLESS lymphadenopathy
50
Marginal Zone Lymphoma
Def'n, B Cell Marker, Assoc?
Neoplastic prolif of small B cells (CD20+) -> Expansion of marginal zone.
('Marginal Zone' formed by post-germinal center B cells)
CD20+.
ASSOC:
- Chronic Infl states (ie Hashimoto's Thyroiditis, Sjogren's Sx, H pylori Gastritis)
51
Burkitt's Lymphoma
Def'n, B Cell Marker, Genetics, Assoc (micro),
Locations (2, based on form), Pres (Epi + 1),
Micro Findings (CLUE + 1)?
Neoplastic prolif of int-sized B cells (CD20+).
CD20+.
C-myc (8;14) translocation.
ASSOC:
- EBV
- Jaw (African form)
- Abdomen / Pelvis (Sporadic form)
PRES: Child / Young Adult
- Extranodal mass
FINDINGS:
- "Starry-sky" appearance (sheets of lymphocytes with interspersed macrophages)
- High mitotic index
52
Diffuse Large B Cell Lymphoma
Def'n, B Cell Marker, Genetics, Etiologies (2),
Pres (Epi + 2 options)?
Neoplastic prolif of large B cells (CD20+) that grow diffusely in "sheets".
CD20+.
(14;18) translocation.
ET:
- Arises sporadically
- Arises from transformation of a low-grade lymphoma (ie follicular lymphoma or CLL). *20% are Mature T Cell in origin*.
PRES: Late Adulthood (however 20% in Children)
- Enlarging lymph node OR Extranodal mass
53
Adult T Cell Lymphoma / Leukemia
Def'n, Assoc (2), Pres (3 possible Epi + 4)?
Neoplastic prolif of mature CD4+ T cells.
ASSOC:
- HTLV-1
- IV Drug Abuse
PRES: MC in Carib, West Africa + Japan
- Generalized lymphadenopathy
- Hepatosplenomegaly
- Skin rash / Cutaneous lesions
- Lytic (punched-out) bone lesions + Hypercalcemia
54
Mycosis Fungoides / Sezary Sx
Def'n, T Cell Marker, Pres (Epi + 2), Micro Findings (2)
Neoplastic prolif of mature CD4+ T cells that infiltrate SKIN.
CD4+
PRES: Adults
- Localized skin rash / nodules / patches / plaques / tumors
('Pautrier Microabscesses': aggregates of neoplastic cells in epidermis)
- Sezary Sx: when neoplastic cells spread to BLOOD
('Sezary Cells': Lymphocytes with cerebriform nuclei)
FINDINGS:
- Pautrier Microabscesses
- Sezary cells
55
Acute Lymphoblastic Leukemia (ALL)
```
Cell Markers (2), Mech, Assoc,
Pres (Epi + 2 options), Comp (2 Mets)?
```
- TdT+ (marker of pre-B + pre-T cells)
- CD10+ (pre-B cells)
MECH:
BM + Peripheral bl replaced by SIGNIFICANTLY↑Lymphoblasts.
ASSOC:
- Down Sx
PRES: Children 5-15
- BM involvement (Children)
- Mediastinal mass (Adolescent Males)
COMP: Spread to:
- CNS
- Testes
56
B-ALL
Cell Markers (3), Prognosis (2)?
TdT+ lymphoblasts that express: CD10, CD19 + CD20.
PROG:
- Excellent response to Chemo. Although requires prophylaxis to CSF + Scrotum.
- Prog based on cytogenetic abnormalities:
- t (9;22) = POOR prog
- t (12;21) = GOOD prog
57
T-ALL
Cell Markers (5), Mech, Pres (Epi + 1)?
Tdt+ lymphoblasts that express CD2, CD3, CD4, CD7 + CD8.
MECH = Leukemic cell infiltration of Thymus.
PRES: Teenagers
- Mediastinal / Thymic mass
58
Acute Myeloid Leukemia (AML)
RF (4), Pres (Epi), Micro Findings (2)?
RF:
- Down Sx
- Myeloproliferative Disorders
- Alkylating Chemo
- Radiation
PRES: 65 yrs = median age of onset
FINDINGS:
- Auer Rods
- ↑circ Myeloblasts
59
M3 AML
Genetics, Pres?
(15;17) translocation.
PRES:
- DIC (can be induced by Chemo -> release of Auer rods)
60
Acute Promyelocytic Leukemia (APL)
Genetics, Comp, RX?
RAR (15;17) translocation.
(=> accumulation of blasts / promyelocytes)
COMP:
- DIC (abnormal promyelocytes c/tn numerous 1RY GRANULES that increase risk for DIC)
RX:
- All-Trans-Retinoic Acid (ATRA, Vit A derivative): (binds altered RAR receptor -> causes promyelocytes to mature and eventually die)
61
Acute Megakaryoblastic Leukemia
Def'n, Assoc?
Neoplastic prolif of megakaryoblasts. Lack MPO.
ASSOC:
- Down Sx: usually arises before age 5
62
Acute Monocytic Leukemia
Def'n, Location?
Neoplastic prolif of monoblasts. Usually lack MPO.
MC = Gums.
63
Myelodysplastic Syndromes
Def'n (3 steps), General Mech, Causes (2),
Micro Findings (2),
Comp (3)?
Stem Cell disorders involving INEFFECTIVE HPS. ->
DEFECTIVE CELL MATURATION of all non-lymphoid lineages ->
HYPERCELLULAR BM.
MECH = Dysplasia
CAUSES:
- De novo mutations
- Envir exposures (ie benzene, chemotherapy, radiation)
FINDINGS:
- ↑Blasts
- Cytopenia
COMP:
- AML (esp with prior exposure to radiation / alkylating agents)
- Bleeding
- Infection
64
Pseudo Pelger-Huet Anomaly
Def'n?
Neutrophils with bilobed nuclei.
| Typically seen after chemotherapy.
65
Chronic Leukemia
Pres (Epi + 1)?
PRES: Older Adults
| - *Insidious onset*
66
Chronic Lymphocytic Leukemia (CLL)
Def'n, Cell Markers (2),
Pres (Epi + 2), Micro Findings (CLUE + 1),
Comp (3)?
Neoplastic prolif of naive B cells that co-express CD5 + CD20.
CD5 + CD20.
PRES: > 60 yrs
- * Slow progression. Often asymptomatic. *
- General lymphadenopathy (lymph node involvement)
FINDINGS:
- "Smudge Cells": WBCs that look smudged
- ↑Lymphocytes
COMP:
- Richter Transformation: Transformation to Diffuse Large B Cell Lymphoma. Marked clinically by enlarging lymph nodes / spleen.
- Warm Ig Autoimmune Hemolytic Anemia
- ↓antibodies -> Infection (*** MCC of Death ***)
67
Hairy Cell Leukemia
Def'n (incl CLUE), Mech, Chars, Pres (CLUE + 1), RX?
Neoplastic prolif of mature B cells char by "hairy / filamentous cytoplasmic processes".
MECH = BM fibrosis
CHARS:
- Tartrate-Resistant Acid Phosphatase (TRAP)+ cells
PRES:
- "Dry tap" on BM Aspiration (due to BM fibrosis)
- Splenomegaly (due to accumulation of hairy cells in red pulp)
RX:
- Cladribine / 2-CDA (adenosine deaminase inhibitor = allows adenosine to accumulate to toxic levels in neoplastic B cells)
68
Myeloproliferative Disorders
Def'n (3 steps), Mech, Pres (Epi), Micro Findings, Comp (3)?
Neoplastic prolif of mature cells of myeloid lineage ->
↑# of cells of myeloid lineage -> HYPERCELLULAR BM.
MECH (*All EXCEPT CML*) = Mutations
PRES: Late Adulthood (50-60 yrs)
FINDINGS:
- High WBC count
COMP:
- Progression to marrow fibrosis
- Acute Leukemia
- ↑risk for Hyperuricemia / Gout (due to high turnover of cells)
69
Chronic Myeloid Leukemia (CML)
Def'n, Genetics, Pres (Epi + 1), Micro Findings (3), Labs,
Comp, RX?
Neoplastic prolif of mature myeloid cells, esp granulocytes and their precursors.
Translocation (9;22) = Philadelphia chr.
PRES: 65 yrs = median age at dx
- Splenomegaly (suggests accel phase of disease)
FINDINGS:
- Band Cells↑
- Neutrophils↑
- Basophils↑
LABS:
- VERY LOW Leukocyte ALP (due to immature granulocytes)
COMP:
- Blast crisis (*** MCC of Death ***)
- Acute Leukemia (*** usually follows splenomegaly ***)
RX:
- Imatinib (blocks tyrosine kinase activity)
70
Essential Thrombocythemia (ET)
Def'n, Genetics, General Pres, Micro Findings (3)?
*** Similar to PV except specific for overproduction of abnormal Platelets ***
Neoplastic prolif of mature myeloid cells, esp PLATELETS.
JAK2 Kinase mutation.
PRES: *Due to↑risk of bleeding and/or thrombosis*
FINDINGS:
- Enlarged Megakaryocytes in BM
- Granulocytes↑
- RBCs↑
71
Polycythemia Vera (PV)
Def'n, Genetics,
Pres (5: 3 + 2 CLUES), Micro Findings (5), Labs (2),
Comp, Prog, RX (2)?
Neoplastic prolif of mature myeloid cells, esp RBCs.
JAK2 Kinase mutation (somatic mutation).
PRES: *Mostly due to hyperviscosity of blood*
- Flushed face (due to congestion)
- Headache
- Blurry vision
- "Itching, esp after bathing" (due to histamine release from↑mast cells)
- *RARE* "Erythromelalgia" (severe burning pain + reddish or bluish coloration. Due to blood clots in extremities)
FINDINGS:
- Plasma Volume↑
- RBC Mass SIGNIFICANTLY↑
- Granulocytes↑
- Mast Cells ↑
- Platelets↑
LABS:
- EPO↓(due to negative feedback)
- Hematocrit > 55%
COMP:
-↑risk of venous thrombosis / 2ry polycythemia (due to natural or artificial↑in EPO levels)
PROG = Death w/in 1 yr without treatment.
RX:
- Phlebotomy = 1st line
- Hydroxyurea = 2nd line
72
Myelofibrosis
Def'n, Genetics, Mech,
Pres, Micro Findings (3: CLUE + 2),
Comp (2)?
Neoplastic prolif of mature myeloid cells, esp MEGAKARYOCYTES.
JAK2 Kinase mutation (50% of cases).
MECH = Fibrotic obliteration of BM.
(due to Megakaryocytes producing excess PDGF)
PRES:
- Splenomegaly (due to extramedullary hps)
FINDINGS:
- "Teardrop" RBCs
- Nucleated RBCs
- Immature Granulocytes
COMP:
- ↑risk of infection
- ↑risk of bleeding / thrombosis
73
Multiple Myeloma
Def'n,
Pres (Epi + 4), Labs, Urine (CLUE), Micro Findings (CLUE + 2),
Appearance on Imaging (XR + SPEP),
Comp (3)?
Malignant prolif of PLASMA CELLS in BM.
Produces large amounts of IgG or IgA.
PRES: > 40-50 yrs
- Bone pain + Hypercalcemia (neoplastic cells activate RANK receptor on osteoclasts -> bone destruction / lytic lesions)
- Amyloidosis (FREE Ig light chains circulate and deposit in tis)
- Anemia
- Renal Insufficiency
LABS:
- IgG or IgA spike (neoplastic plasma cells produce Ig)
URINE:
- Bence Jones proteins (FREE Ig light chain excreted in urine)
FINDINGS:
- Monoclonal Plasma cells with "fried-egg" appearance, "clockface chromatin" + intracytoplasmic inclusions c/tning Ig.
- Rouleaux Formation of RBCs
- Russell Bodies
IMAGING:
- XR: "Lytic" / "punched-out" bone lesions, esp in skull + vert
- SPEP: 'M Spike' (due to monoclonal IgG or IgA)
COMP:
- ↑fracture risk
- "Myeloma Kidney" -> Renal Failure (FREE Ig light chain deposition in kidney tubules)
- Infection = MCC Death (monoclonal Igs lack antigenic diversity)
74
Monoclonal Gammopathy of Undetermined Significance (MGUS)
Def'n, Pres (Epi), Micro Findings (CLUE), Comp?
Monoclonal Plasma cell expansion WITHOUT symptoms of MM.
PRES: Elderly
*** Asymptomatic! ***
FINDINGS:
- BM w
75
Waldenstrom Macroglobulinemia
Def'n, Pres (3), Urine (CLUE), Imaging, RX?
B cell lymphoma with monoclonal IgM production.
FINDINGS:
- Generalized lymphadenopathy
- Visual + Neuro defects (eg retinal hem, stroke)
- Bleeding (IgM = large pentamer -> serum hyperviscosity -> defective platelet aggregation)
URINE:
- Bence Jones proteins in urine
IMAGING:
- SPEP:↑serum protein + 'M Spike' (due to monoclonal IgM)
RX:
- Plasmapheresis (removes IgM from serum)
76
Bruton Disease
Def'n, Pres (Epi + 1)?
X-linked agammaglobulinemia. NO mature B cells.
PRES: Male child
- Recurrent infections
77
Langerhans Cell Histiocytosis
Def'n, Cell Markers (2), Pres (Epi + 3), Micro Findings (CLUE)?
Neoplastic prolif of Langerhans cells.
Cells are functionally immature and don't efficiently stim T cells via antigen presentation.
CD1a + S100 cells.
PRES: Child
- Skin rash
- Lytic bone lesions
- Recurrent Otitis Media with mass involving Mastoid bone
FINDINGS:
- Birbeck / "Tennis racket" granules
78
Letterer-Siwe Disease
Pres (Epi + 2)?
PRES: Infant
79
Eosinophilic Granuloma
Def'n + Location, Pres (Epi + 1), Micro Findings?
Benign prolif of Langerhans cells in BONE.
PRES: Adolescents
- Pathologic bone fracture *** Skin NOT involved ***
FINDINGS: (Biopsy)
- Langerhans cells + mixed infl cells, incl numerous Eosinophils
80
Hand-Schuller Christian Dz
Pres (Epi + Tetrad)?
PRES: Child
1. Scalp rash
2. Lytic skull defects
3. Exophthalmos
4. Diabetes Insipidus
81
Babesiosis
CB + Source,
Epi (Geo), Pres, Micro Finding,
RX (2)?
Babesia- Ixodes tick.
EPI: Northeastern U.S
PRES: *Asplenia↑risk of severe dz*
- Fever + Hemolytic Anemia
FINDING: Ring + "Maltese Cross" on bl smear
RX: Atovaquone + Azithromycin
82
Parvovirus
Possible Pres (4)?
PRES:
- B19 virus -> Aplastic Crises (in Sickle Cell dz)
- RBC destruction -> Hydrops Fetalis + death (in fetus)
- Erythema Infectiosum (5th Dz) -> "Slapped cheeks" rash (in children)
- RBC aplasia + RA-like symptoms (in adults)
83
Kaposi Sarcoma
CB, Def'n, Epi (2)?
HHV-8.
Neoplasm of endothelial cells.
EPI:
- HIV / AIDS patients
- Transplant patients
