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POS 1: HEMATO > 3. Síndrome Leucémico-Mieloma Múltiple > Flashcards

3. Síndrome Leucémico-Mieloma Múltiple Flashcards

1
Q

¿Qué es la leucemia?

A

Enfermedad circulante en sangre periférica

Se pueden observar células enfermas en un análisis de sangre

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2
Q

¿Cómo se dividen las leucemias según su agudeza?

A
  • Aguda (LMA o LLA)
  • Crónica (LMC o LLC)

La leucemia aguda se caracteriza por células inmaduras, mientras que la crónica por células maduras.

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3
Q

¿Qué tipo de células se acumulan en la leucemia aguda?

A

Células inmaduras (blastos)

Se infiltran en la médula ósea en más de un 20%

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4
Q

¿Qué tipo de células se acumulan en la leucemia crónica?

A

Células maduras (neutrófilos, basófilos,…)

Estas células son más desarrolladas en comparación con las de la leucemia aguda.

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5
Q

Clasificación de las leucemias según la estirpe celular

A
  • Mieloide (LMA o LMC)
  • Linfoide (LLA o LLC)

Esta clasificación se basa en el tipo de célula que prolifera.

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6
Q

¿Qué es la leucemia linfoblástica aguda?

A

Proliferación de células inmaduras de estirpe linfoide en sangre periférica

Es un tipo específico de leucemia aguda.

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7
Q

Definición de leucemia aguda (LMA)

A

Proliferación clonal de células inmaduras (blastos) que infiltran la médula ósea

Debe haber más de un 20% de blastos en la médula ósea.

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8
Q

Etiología de la leucemia aguda

A
  • Radiaciones
  • Sustancias químicas (benceno)
  • Virus (HTLV 1, VEB, VIH)
  • Factores constitucionales (Síndrome de Down, Síndrome de Noonan)
  • Tratamientos de quimioterapia y/o radioterapia
  • Enfermedades hematológicas

La etiología generalmente es desconocida, pero se han identificado varios factores de riesgo.

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9
Q

Clasificación de la leucemia aguda según la célula que prolifera

A
  • Mieloblásticas (mieloblastos)
  • Linfoblásticas (linfoblastos)
  • Bifenotípicas

La mieloblástica es más habitual en adultos, mientras que la linfoblástica es más común en niños.

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10
Q

¿Qué son las leucemias primarias y secundarias?

A
  • Primarias o de novo
  • Secundarias

Se refiere a la origen y desarrollo de la leucemia.

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11
Q

¿Cuáles son los síntomas constitucionales de la leucemia aguda?

A

Astenia, debilidad, pérdida de peso, sudoración

Estos síntomas son comunes en muchas enfermedades hematológicas y reflejan un estado general de mala salud.

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12
Q

¿Qué síntomas se producen por la infiltración de la médula ósea en la leucemia aguda?

A

Anemia, leucopenia, trombopenia, infecciones de repetición, hemorragias

La presencia de blastos en la médula ósea impide la producción de células sanguíneas maduras.

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13
Q

¿Cómo se manifiesta la infiltración de otros órganos en la leucemia aguda?

A

Infiltración de la piel, hígado, bazo (hepatoesplenomegalia), SNC, testículos

La infiltración de estos órganos puede causar síntomas específicos según el órgano afectado.

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14
Q

¿Cuál es la evolución de los síntomas en la leucemia aguda?

A

Desarrollo en un tiempo corto, evolución aguda de los síntomas

El paciente puede morir en semanas si no recibe tratamiento adecuado.

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15
Q

¿Cuál es la principal diferencia clínica entre LMA y LLA?

A

LMA tiene más tropismo por la piel y órganos linfoides; LLA presenta más tropismo por el SNC

Esto afecta la presentación clínica y el enfoque diagnóstico.

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16
Q

¿Cuáles son algunos de los síntomas clínicos de LMA?

A

Infiltración en piel, mucosas, hepatoesplenomegalia

Especialmente en subtipos M4 y M5 de leucemia mieloide aguda.

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17
Q

¿Cuáles son algunos de los síntomas clínicos de LLA?

A

Dolor óseo, infiltración SNC, adenopatías

Estos síntomas reflejan la afectación del sistema linfático y el sistema nervioso central.

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18
Q

¿Qué se observa en el hemograma de un paciente con LMA y LLA?

A

Leucocitosis (LMA) o linfocitosis (LLA) a expensas de blastos

La presencia de blastos en sangre periférica es un hallazgo clave en el diagnóstico de leucemia.

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19
Q

¿Qué se determina mediante el medulograma en el diagnóstico de leucemia?

A

Presencia de más de 20% de blastos en médula ósea

La tinción panóptica es utilizada para visualizar esta presencia.

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20
Q

¿Qué indica una leucocitosis superior a 100.000 leucocitos/uL en leucemia aguda?

A

Es un dato clásico de la leucemia aguda

La leucocitosis es generalmente a expensas de blastos, y puede haber neutropenia en casos de LMA.

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21
Q

¿Qué análisis se realiza para determinar la estirpe de los blastos en leucemia?

A

Citometría de flujo

Este análisis permite clasificar los blastos como mieloides o linfoides, lo cual es crucial para el tratamiento.

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22
Q

La leucemia aguda generalmente cursa con _______.

A

Pancitopenia

Esto se debe a la afectación de la línea mieloide que impacta en la producción de todas las series sanguíneas.

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23
Q

What is the primary treatment for Acute Lymphoblastic Leukemia (LLA) in children?

A

High-dose chemotherapy

Transplantation of hematopoietic precursors is usually reserved for cases of relapse.

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24
Q

What is the typical treatment approach for Acute Myeloid Leukemia (LMA) in adults?

A

High-dose chemotherapy and often hematopoietic precursor transplantation

LMA occurs more frequently in adults compared to LLA.

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25
Q

What are gammapatías monoclonales?

A

A heterogeneous group of diseases characterized by abnormal proliferation of a clone of plasma cells

They are generally associated with the presence of a monoclonal component in serum or urine.

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26
Q

Where are plasma cells normally found in the body?

A

Bone marrow

Plasma cells are not typically seen in large proportions in peripheral blood under normal conditions.

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27
Q

What indicates the presence of leukemia in relation to plasma cells?

A

Detection of plasma cells in peripheral blood

This is indicative of plasma cell leukemia.

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28
Q

What is the clinical spectrum of gammapatías monoclonales?

A
  1. Normal plasma cell
  2. Monoclonal gammopathy of uncertain significance (MGUS)
  3. Malignant transformation (≥ 10% in bone marrow): Multiple Myeloma (MM)

The previous states do not meet the criterion of more than 10% plasma cells.

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29
Q

What are the two groups within the diagnosis of Multiple Myeloma (MM)?

A
  • Asymptomatic Multiple Myeloma
  • Symptomatic Multiple Myeloma

Asymptomatic patients have more than 10% plasma cells in bone marrow but no organ damage.

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30
Q

What characterizes Plasma Cell Leukemia?

A

Presence of plasma cells in peripheral blood

This is a late-stage diagnosis and can be primary or secondary.

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31
Q

Fill in the blank: Gammapatías indicate a _______ of plasma cells.

A

clonal proliferation

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32
Q

True or False: MGUS meets the diagnostic criteria for Multiple Myeloma.

A

False

MGUS is characterized by an immortalized clone of plasma cells but does not meet the diagnostic criteria for MM.

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33
Q

What is the serum level of monoclonal component (CM) in smoldering multiple myeloma (SMM)?

A

≥ 3 g/dL and/or 10% - 60% of plasma cells (CP) in bone marrow (MO)

SMM is characterized by the absence of symptoms and organ damage.

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34
Q

What are the criteria for symptomatic multiple myeloma (MM)?

A

≥ 10% of plasma cells (CP) in MO or biopsy showing plasmacytoma and presence of MDE

MDE stands for myeloma defining events.

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35
Q

Define CRAB in the context of multiple myeloma diagnosis.

A

Hypercalcemia, renal failure, anemia, bone lesions

These criteria indicate tissue damage attributable to monoclonal gammopathy.

36
Q

What characterizes the diagnosis of MGUS (Monoclonal Gammopathy of Undetermined Significance)?

A

CM < 3 g/dL and % of CP in MO < 10%

MGUS does not involve organ damage (no CRAB symptoms).

37
Q

What is the significance of the level of plasma cells in bone marrow for diagnosing MM?

A

CP > 10% indicates MM

This is a critical threshold for diagnosis.

38
Q

What is the relationship between plasma cells in MO and monoclonal component in serum?

A

Higher number of plasma cells in MO correlates with higher monoclonal component in serum

Generally, as CP increases, so does the accumulation of IgG in blood.

39
Q

What is the threshold for the presence of malignancy biomarkers in early MM?

A

At least one of the following:
* Clonal plasma cells in MO ≥ 60%
* FLC ratio > 100
* More than one focal lesion on MRI

These biomarkers help in assessing the malignancy of MM.

40
Q

True or False: In GMSI, plasma cell percentage in MO is greater than or equal to 10%.

A

False

In GMSI, plasma cells in MO are less than 10%.

41
Q

Fill in the blank: The presence of _______ is indicative of symptomatic MM.

A

MDE

MDE refers to myeloma defining events which signify damage.

42
Q

What are the levels of plasma cells (CP) and monoclonal component (CM) in GMSI?

A

CP < 10% and CM < 3 g/dL

GMSI does not present with CRAB symptoms.

43
Q

What is the most commonly found monoclonal component in multiple myeloma?

A

IgG kappa

The exact reason for its prevalence is not known.

44
Q

What are the two groups based on organ involvement in multiple myeloma (MM)?

A
  1. Without organ damage (asymptomatic MM or smoldering MM, without CRAB)
  2. With organ involvement (symptomatic or active MM, with CRAB)

CRAB refers to hypercalcemia, renal insufficiency, anemia, and bone lesions.

45
Q

What does CRAB stand for in the context of symptomatic multiple myeloma?

A

Hypercalcemia, renal insufficiency, anemia, and bone lesions

These are the criteria that indicate organ damage.

46
Q

What is the diagnostic criterion for symptomatic multiple myeloma?

A

Clonal plasma cell infiltration of 10% or more in bone marrow or biopsy, or extramedullary plasmacytoma

If there is a disparity between both methods, the one with higher values is chosen.

47
Q

List one event that defines multiple myeloma.

A
  1. Monoclonal protein in serum (IgG or IgA > 30 g/L)
  2. Organ damage attributed to plasma cell proliferation
  3. Hypercalcemia > 11.5 mg/dL
  4. Monoclonal protein in urine ≥ 500 mg/24 hours
  5. Renal insufficiency: Creatinine > 2 mg/dL or clearance < 40 ml/min
  6. Anemia: Hemoglobin < 10 g/dL or < 2 g/dL below normal
  7. Bone lesions diagnosed by imaging

At least one of these events is required for the diagnosis.

48
Q

What are the criteria for asymptomatic multiple myeloma (smoldering)?

A

Absence of CRAB events or amyloidosis

Both criteria must be met for the diagnosis.

49
Q

What are biomarkers of malignancy in multiple myeloma?

A
  1. Clonal plasma cells in bone marrow
  2. Compromised light chain ratio ≥ 100
  3. More than 1 focal lesion in MRI (greater than 5 mm)

These biomarkers help assess the risk of progression.

50
Q

What is the significance of the light chain ratio in multiple myeloma?

A

A compromised light chain ratio ≥ 100 indicates a higher risk of progression to symptomatic MM

Also, the presence of medullary lesions on MRI increases the risk.

51
Q

True or False: All plasma cells produce heavy and light chains to assemble immunoglobulin.

A

True

They always produce an excess of light chains.

52
Q

What is the average age at diagnosis for multiple myeloma?

A

60 years

Approximately 15% of cases are diagnosed in individuals under 50.

53
Q

What laboratory tests are indicative of multiple myeloma?

A
  1. Increased monoclonal component in blood/urine
  2. Immunofixation to determine specific immunoglobulin
  3. Possible anemia
  4. Possible hypercalcemia
  5. Possible renal insufficiency

These tests help in confirming the diagnosis.

54
Q

What is the minimum percentage of plasma cells in the bone marrow required for a diagnosis of multiple myeloma?

A

> 10% of plasma cells in bone marrow

This is assessed through a medullogram.

55
Q

What imaging test can reveal lytic lesions in multiple myeloma?

A

CT scan of the whole body

This imaging technique is used to identify bone lesions.

56
Q

¿Qué manifestaciones clínicas se presentan por la acumulación de células plasmáticas y componente monoclonal?

A
  • Fallo renal
  • Hiperviscosidad de la sangre
  • Amiloidosis
57
Q

¿Cómo se relaciona la disminución de inmunoglobulinas normales con la inmunodeficiencia?

A

La presencia de mucha IgG kappa y menos de las demás inmunoglobulinas provoca poca policlonalidad, lo que facilita infecciones

58
Q

¿Qué consecuencias puede tener la infiltración de médula ósea?

A
  • Anemia
  • Destrucción ósea
  • Daño neurológico
  • Hipercalcemia
  • Dolor óseo
59
Q

¿Qué es el componente monoclonal en el contexto de las manifestaciones clínicas?

A

Es una proteína que se acumula anormalmente, contribuyendo a condiciones como amiloidosis y hiperviscosidad

60
Q

¿Qué indica un proteinograma normal?

A

Habrá pico de albúmina y el patrón usual de cada inmunoglobulina, conocido como ‘mano de surfero’

61
Q

¿Qué se observa en un proteinograma de mieloma múltiple?

A

Pico anormal en gamma que representa el componente monoclonal aumentado

62
Q

¿Qué es la hiperviscosidad de la sangre?

A

Un aumento en la viscosidad sanguínea que puede resultar de la acumulación de proteínas anormales

63
Q

¿Qué es la amiloidosis?

A

Pliegue anómalo de las proteínas que se depositan en lugares inadecuados, en este caso, inmunoglobulinas

64
Q

¿Qué puede causar la liberación de calcio en pacientes con infiltración de médula ósea?

A

Destrucción ósea

65
Q

¿Qué tipo de infecciones son comunes en pacientes con disminución de inmunoglobulinas?

A

Infecciones debido a inmunodeficiencia

66
Q

¿Qué análisis se realiza para determinar el componente monoclonal acumulado?

A

Inmunofijación

67
Q

¿Qué proteínas se representan en un proteinograma?

A
  • Albúmina
  • Cadenas alfa 1
  • Cadenas alfa 2
  • Cadenas beta
  • Cadenas gamma de las inmunoglobulinas
68
Q

¿Verdadero o falso? La hipercalcemia puede ser causada por la destrucción ósea en pacientes con mieloma múltiple.

A

Verdadero

69
Q

Fill in the blank: La _______ es una condición que se presenta por la acumulación de inmunoglobulinas en la sangre.

A

hiperviscosidad

70
Q

¿Qué síntomas pueden resultar de la destrucción ósea en pacientes con mieloma múltiple?

A
  • Dolor óseo
  • Daño neurológico
71
Q

¿Qué representa un pico anormal en gamma en un proteinograma?

A

El componente monoclonal aumentado

72
Q

What does the test for INMUNOFIJACIÓN determine?

A

It determines which immunoglobulin is accumulating

The test involves mixing the sample with Anti-IgG, Anti-IgA, etc. to detect the specific immunoglobulin.

73
Q

What is the most common immunoglobulin found in INMUNOFIJACIÓN?

A

IgG kappa

If there is polyclonality and an excess of all immunoglobulins, it is not multiple myeloma (MM).

74
Q

What is ‘inmunoparesia’ in the context of multiple myeloma?

A

A decrease in the rest of immunoglobulins

This condition leads to immunosuppression and recurrent infections.

75
Q

How is the progression of immunoglobulin peaks used in multiple myeloma treatment?

A

To monitor the treatments

Monitoring does not require monthly bone marrow punctures; blood samples and proteinograms suffice.

76
Q

What indicates that the treatment for multiple myeloma is functioning?

A

A decrease in the amount of light chains

The quantity of light chains parallels the number of plasma cells.

77
Q

Where might the monoclonal component be detected if not in blood?

A

In urine

Typically, these components are only light chains, as they are the most eliminated.

78
Q

What renal damage is associated with multiple myeloma?

A

Kidney damage due to light chain elimination

This long-term effect is often referred to as ‘myeloma kidney’.

79
Q

What are the main components of the treatment for multiple myeloma?

A

Chemotherapy + autologous transplant

The treatment involves chemotherapy to reduce plasma cells, followed by the infusion of healthy hematopoietic progenitor cells.

80
Q

What is the purpose of administering colony-stimulating factor injections after chemotherapy?

A

To release hematopoietic CD34+ cells

These healthy progenitor cells are collected from peripheral blood.

81
Q

What is the role of autologous transplant in multiple myeloma treatment?

A

To replenish the bone marrow

The patient’s own progenitor cells are returned to the bone marrow, which takes about 12 days to recover levels.

82
Q

What does an allogeneic transplant involve in the treatment of multiple myeloma?

A

Using stem cells from a compatible donor

If a compatible sibling is available, their bone marrow can be injected to establish a new immune system.

83
Q

What is a potential benefit of an allogeneic transplant?

A

Prevention of relapses

The graft-versus-host effect can be beneficial in eliminating residual multiple myeloma plasma cells.

84
Q

Is there a curative treatment for multiple myeloma?

A

No curative treatment exists

However, there are medications available for managing multiple myeloma throughout the patient’s life.

85
Q

How long does aplasia last after treatment, and what is the associated risk?

A

30-40 days, with a risk of infections

This risk necessitates the use of autologous or allogeneic transplants to restore bone marrow quickly.

POS 1: HEMATO (10 decks)

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