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Classic Presentations > HEME > Flashcards

HEME Flashcards

1
Q

ANTI-Coagulation Pathologies (4)

A
  • Hemophilia A + B
  • Vitamin K Deficiency
  • DIC
  • Von Willebrand’s Disease
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2
Q

Disorders of Primary Hemostasis (Platelets)

2 General Clinical Features?

A
  1. Mucosal Bleeding

2. Skin Bleeding

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3
Q

Mucosal Bleeding

Pres (6)?

A

PRES:

    • Epistaxis (nose bleed)
  • Hemoptysis
  • Hematuria
  • Menorrhagia (heavy / prolonged periods)
  • GI bleeding
  • Intracranial bleeding (severe cases only)
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4
Q

Skin Bleeding

Pres (2)?

A

PRES:

  • Petechiae / Purpura / Ecchymoses
  • Easy Bruising
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5
Q

Petechia

Def’n, Causes (2)?

A

Small red or purple spots on skin caused by minor hemorrhage /
broken capillaries under skin.

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6
Q

Purpura

Def’n?

A

Petechia 3-10 mm.

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7
Q

Ecchymoses

Def’n?

A

Petechia > 1 cm.

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8
Q

PRO-Coagulation Pathologies (4)

A
  • Protein C/S Deficiency
  • Antithrombin Deficiency
  • Prothrombin Mutation
  • Factor 5 Leiden
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9
Q

Thrombotic Thrombocytopenic Purpura (TTP)

Pres (Pentad), Micro Finding?

A

PRES:

  1. Microangiopathic Hemolytic Anemia
  2. Thrombocytopenia
  3. Fever
  4. Neuro symptoms (thrombi involve CNS vessels)
  5. Renal symptoms (thrombi involve Renal vessels)

FINDING:
- Schistocytes

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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

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10
Q

Hemolytic Uremic Sx (HUS)

Mech, Causative Bugs (2)?

A

MECH = Toxin enhances cytokine release

CB:

  • EHEC (-> Shiga-like toxin)
  • Shigella (-> Shiga toxin)
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11
Q

Disorders of Secondary Hemostasis (Coagulation)

2 General Clinical Features?

A
  1. Hemarthrosis (bleeding into joints + muscles)

2. Rebleeding after surgical procedures

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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
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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

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14
Q

Raynaud Phenomenon

Def’n, Pres, Seen In?

A

Vasospasm in extremities.

PRES = Pale, cold + painful fingers / toes

SEEN IN:
- SLE

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15
Q

Rouleaux Formation

Def’n, Mech, Seen In?

A

Stacks of RBCs.

MECH = due to↑ESR

SEEN IN:
- Multiple Myeloma

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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
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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
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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 ↑
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19
Q

“Target Cells +↓Haptoglobin”

Classification, Micro Findings (2), Labs (2), Urine?

A

INTRAvascular Hemolytic Anemia.

FINDINGS:

  • ↑Reticulocytes
  • Schistocytes

LABS:

  • Haptoglobin ↓
  • LDH ↑

URINE:
- Hb

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20
Q

“Hemolytic Anemia in newborn”

A

PK Deficiency (EXTRAvascular Hemolytic Normocytic Anemia)

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21
Q

“Inherited defect of RBC cytoskeleton-memb tethering proteins
(MC involving ankyrin, band + spectrin)” ->
MEMBRANE BLEBS”

A

Hereditary Spherocytosis

EXTRAvascular Hemolytic Normocytic Anemia

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22
Q

“Red urine in morning”

A

PNH

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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

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24
Q

Iron Deficiency Anemia

Causes (4), Pres (3, incl 1 Triad)?

A

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
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25
Q

Anemia of Chronic Disease (ACD)

Etiologies, Micro Finding, Labs, Course?

A

ET:
- Chronic infl

FINDINGS:
- Basophilic stippling

LABS:
- Hepcidin↑

COURSE = Can become MICROcytic in long-standing disease.

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26
Q

β-Thalassemia

Micro Findings (5: 3 general + 2 specific cell types)?

A

FINDINGS:

  • Anisocytosis
  • Poikilocytosis
  • Hypochromia
  • Schistocytes
  • Target cells
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27
Q

β-Thalassemia Major (Homozygote)

Pres (3, incl 1 CLUE), Appearance on XR (CLUE)?

A

PRES:

  • “Chipmunk facies”
  • Severe anemia requiring bl transfusion (2ry hemochromatosis)
  • Skeletal deformities (due to marrow expansion)

XR:
- “Crew cut” / “hair on end” appearance

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28
Q

Sideroblastic Anemia

Micro Findings (3), RX?

A

FINDINGS:

  • Ringed Sideroblasts
  • Pappenheimer Bodies
  • Iron-laden mitochondria

RX = Vit B6

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29
Q

Cold Agglutinin Disease

Def’n, Seen In (3), Pres (CLUE)?

A

Autoimmune (IgM) ACUTE hemolytic anemia triggered by cold.

SEEN IN:

  • CLL
  • Mycoplasma pneumonia infections
  • Infectious Mononucleosis

PRES:
- “BLUE painful fingers / toes”

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30
Q

G6PD Deficiency

Def’n, Mech (3 steps), Inher, Causative Agents (5),
Pres (Epi + CLUE + 1), Micro Findings (2)?

A

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
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31
Q

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Mech, Assoc, Pres (CLUE + Triad), Comp?

A

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

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32
Q

Warm Agglutinin Disease

Def’n, Seen In (3: 2 dz + 1 drug), Micro Finding?

A

Autoimmune (IgG) CHRONIC hemolytic anemia. Coombs+.

SEEN IN:

  • CLL
  • SLE
  • a-methyldopa

FINDING:
- Spherocytes

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33
Q

PK Deficiency

Pres (CLUE), Micro Finding?

A

PRES = “Hemolytic Anemia in a newborn”

FINDING:
- Schistocytes

34
Q

HbC Defect

Micro Finding?

A

FINDING:

- Target Cells

35
Q

Hereditary Spherocytosis

Pres (2), Labs, RX?

A

PRES:

  • Splenomegaly
  • Aplastic crisis (Parvo B19 infection)

LABS:
- Osmotic Fragility Test +

RX = Splenectomy

36
Q

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)?

A

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
Q

Angiopathic Hemolytic Anemias

Mech, Micro Finding?

A

MECH = Mechanical destruction of RBCs

FINDING = Schistocytes

38
Q

Microangiopathic Hemolytic Anemia

Seen In (4: 2 heme + 2 other)?

A

SEEN IN:

  • Malignant Htn
  • SLE
  • DIC
  • TTP / HUS
39
Q

Macrocytic Megaloblastic Anemias

Micro Findings (2)?

A

FINDINGS:

  • Macro-Ovalocytes
  • Hypersegmented Neutrophils
40
Q

Pernicious Anemia

Causes?

A

CAUSES:

- Autoimmune / Chronic Atrophic Gastritis

41
Q

PAINLESS Lymphadenopathy (LAD)

Seen In (3)?

A

SEEN IN:

  • Chronic infl
  • Lymphoma
  • Metastatic Carcinoma
42
Q

Follicular Hyperplasia (B cell region)

Seen In (2)?

A

SEEN IN:

  • Early HIV
  • RA
43
Q

Paracortex Hyperplasia (T cell region)

Seen In?

A

SEEN IN:

- Viral infections

44
Q

B SYMPTOMS of Lymphoma (3)

A
  • Fever (low-grade)
  • Weight Loss
  • Night sweats

** Same as TB **

45
Q

Nodular Sclerosis

Pres (CLUE), Micro Finding (CLUE)?

A

PRES = “Enlarging cervical or mediastinal lymph node in a young adult (usually female)”

FINDING:
- RS cells present in “lake-like” / “lacunar” spaces

46
Q

Lymphocyte-Depleted HL

Seen In (2 Epi)?

A

SEEN IN:

  • Elderly
  • HIV + indivs
47
Q

Mixed Cellularity HL

Micro Finding?

A

FINDINGS:

- Abundant Eosinophils (RS cells produce IL-5)

48
Q

Follicular Lymphoma

Def’n, B Cell Marker, Genetics, Pres (Epi + CLUE), RX, Comp?

A

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
Q

Mantle Cell / Zone Lymphoma

Def’n, B Cell Markers (2), Genetics, Pres (Epi + 1)?

A

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
Q

Marginal Zone Lymphoma

Def’n, B Cell Marker, Assoc?

A

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
Q

Burkitt’s Lymphoma

Def’n, B Cell Marker, Genetics, Assoc (micro),
Locations (2, based on form), Pres (Epi + 1),
Micro Findings (CLUE + 1)?

A

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
Q

Diffuse Large B Cell Lymphoma

Def’n, B Cell Marker, Genetics, Etiologies (2),
Pres (Epi + 2 options)?

A

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
Q

Adult T Cell Lymphoma / Leukemia

Def’n, Assoc (2), Pres (3 possible Epi + 4)?

A

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
Q

Mycosis Fungoides / Sezary Sx

Def’n, T Cell Marker, Pres (Epi + 2), Micro Findings (2)

A

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
Q

Acute Lymphoblastic Leukemia (ALL)

Cell Markers (2), Mech, Assoc,
Pres (Epi + 2 options), Comp (2 Mets)?
A
  • 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
Q

B-ALL

Cell Markers (3), Prognosis (2)?

A

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
Q

T-ALL

Cell Markers (5), Mech, Pres (Epi + 1)?

A

Tdt+ lymphoblasts that express CD2, CD3, CD4, CD7 + CD8.

MECH = Leukemic cell infiltration of Thymus.

PRES: Teenagers
- Mediastinal / Thymic mass

58
Q

Acute Myeloid Leukemia (AML)

RF (4), Pres (Epi), Micro Findings (2)?

A

RF:

  • Down Sx
  • Myeloproliferative Disorders
  • Alkylating Chemo
  • Radiation

PRES: 65 yrs = median age of onset

FINDINGS:

  • Auer Rods
  • ↑circ Myeloblasts
59
Q

M3 AML

Genetics, Pres?

A

(15;17) translocation.

PRES:
- DIC (can be induced by Chemo -> release of Auer rods)

60
Q

Acute Promyelocytic Leukemia (APL)

Genetics, Comp, RX?

A

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
Q

Acute Megakaryoblastic Leukemia

Def’n, Assoc?

A

Neoplastic prolif of megakaryoblasts. Lack MPO.

ASSOC:
- Down Sx: usually arises before age 5

62
Q

Acute Monocytic Leukemia

Def’n, Location?

A

Neoplastic prolif of monoblasts. Usually lack MPO.

MC = Gums.

63
Q

Myelodysplastic Syndromes

Def’n (3 steps), General Mech, Causes (2),
Micro Findings (2),
Comp (3)?

A

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
Q

Pseudo Pelger-Huet Anomaly

Def’n?

A

Neutrophils with bilobed nuclei.

Typically seen after chemotherapy.

65
Q

Chronic Leukemia

Pres (Epi + 1)?

A

PRES: Older Adults

- Insidious onset

66
Q

Chronic Lymphocytic Leukemia (CLL)

Def’n, Cell Markers (2),
Pres (Epi + 2), Micro Findings (CLUE + 1),
Comp (3)?

A

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
Q

Hairy Cell Leukemia

Def’n (incl CLUE), Mech, Chars, Pres (CLUE + 1), RX?

A

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
Q

Myeloproliferative Disorders

Def’n (3 steps), Mech, Pres (Epi), Micro Findings, Comp (3)?

A

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
Q

Chronic Myeloid Leukemia (CML)

Def’n, Genetics, Pres (Epi + 1), Micro Findings (3), Labs,
Comp, RX?

A

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
Q

Essential Thrombocythemia (ET)

Def’n, Genetics, General Pres, Micro Findings (3)?

A

** 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
Q

Polycythemia Vera (PV)

Def’n, Genetics,
Pres (5: 3 + 2 CLUES), Micro Findings (5), Labs (2),
Comp, Prog, RX (2)?

A

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
Q

Myelofibrosis

Def’n, Genetics, Mech,
Pres, Micro Findings (3: CLUE + 2),
Comp (2)?

A

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
Q

Multiple Myeloma

Def’n,
Pres (Epi + 4), Labs, Urine (CLUE), Micro Findings (CLUE + 2),
Appearance on Imaging (XR + SPEP),
Comp (3)?

A

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
Q

Monoclonal Gammopathy of Undetermined Significance (MGUS)

Def’n, Pres (Epi), Micro Findings (CLUE), Comp?

A

Monoclonal Plasma cell expansion WITHOUT symptoms of MM.

PRES: Elderly
** Asymptomatic! **

FINDINGS:
- BM w

75
Q

Waldenstrom Macroglobulinemia

Def’n, Pres (3), Urine (CLUE), Imaging, RX?

A

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
Q

Bruton Disease

Def’n, Pres (Epi + 1)?

A

X-linked agammaglobulinemia. NO mature B cells.

PRES: Male child
- Recurrent infections

77
Q

Langerhans Cell Histiocytosis

Def’n, Cell Markers (2), Pres (Epi + 3), Micro Findings (CLUE)?

A

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
Q

Letterer-Siwe Disease

Pres (Epi + 2)?

A

PRES: Infant

79
Q

Eosinophilic Granuloma

Def’n + Location, Pres (Epi + 1), Micro Findings?

A

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
Q

Hand-Schuller Christian Dz

Pres (Epi + Tetrad)?

A

PRES: Child

  1. Scalp rash
  2. Lytic skull defects
  3. Exophthalmos
  4. Diabetes Insipidus
81
Q

Babesiosis

CB + Source,
Epi (Geo), Pres, Micro Finding,
RX (2)?

A

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
Q

Parvovirus

Possible Pres (4)?

A

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
Q

Kaposi Sarcoma

CB, Def’n, Epi (2)?

A

HHV-8.

Neoplasm of endothelial cells.

EPI:

  • HIV / AIDS patients
  • Transplant patients

Classic Presentations (18 decks)

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